Innovative therapeutics

Investors

BioMarin Announces Second Quarter 2008 Financial Results
Second Quarter Profit Driven by Strong Revenue Growth Improved Total Revenue Guidance
Conference Call and Webcast to Be Held Today at 5:00 p.m. ET (22:00 CET)
Aug 5, 2008
PRNewswire-FirstCall
NOVATO, Calif.
  Financial Highlights ($ in millions, except per share data)

  Item                                     Q2 2008        Q2 2007 Comparison
  Total BioMarin Revenue                   $64.2          122% increase
  Naglazyme Net Product Revenue            $35.1          67.9% increase
  Aldurazyme Net Sales by Genzyme          $38.7          33.0% increase
  Aldurazyme BioMarin Net Product Revenue  $13.4          NA
  Kuvan Net Product Revenue                $12.0          NA
  GAAP Net Income (Loss)                   $3.8, $0.04   ($3.9), ($0.04)
                                            per share      per share
  Non-GAAP Net Income (Loss)               $9.7, $0.10    $0.4, $0.00
                                            per share      per share

  2008 Guidance

  Item                                        Updated Guidance  Previous
                                                                Guidance
  Total BioMarin Revenue*                     $288 to $326      $271 to $316
  Total Net Product Revenue                   $247 to $285      $230 to $275
  Naglazyme Net Product Revenue               $130 to $140      $115 to $125
  Aldurazyme Net Product Sales by Genzyme     Unchanged         $135 to $145
  Aldurazyme Net Product Revenue to BioMarin  $72 to $80        $70 to $80
  Kuvan Net Product Revenue                   $45 to $65        $45 to $70
  Net Income (GAAP)*                          $30 to $42        $28 to $40
  Net Income (non-GAAP)*                      $54 to $69        $52 to $67

*Assumes that the $30 million milestone for EU Kuvan approval will be earned in 2008

BioMarin Pharmaceutical Inc. (Nasdaq and SWX: BMRN) today announced financial results for the second quarter ended June 30, 2008. Net income was $3.8 million ($0.04 per share) for the second quarter of 2008, compared to a net loss of $3.9 million ($0.04 per share) for the second quarter of 2007. Non-GAAP net income was $9.7 million ($0.10 per share) for the second quarter of 2008, compared to non-GAAP net income of $0.4 million ($0.00 per share) for the second quarter of 2007. Non-GAAP net income/loss excludes non-cash stock compensation expense, which was $5.9 million for the three months ended June 30, 2008, compared to $4.3 million for the three months ended June 30, 2007. Net income for the six months ended June 30, 2008 was $5.5 million ($0.06 per share), compared to a net loss of $13.2 million ($0.14 per share) for the six months ended June 30, 2007. Non-GAAP net income was $15.9 million ($0.16 per share) for the six months ended June 30, 2008, compared to non-GAAP net loss of $5.4 million ($0.06 per share) for the six months ended June 30, 2007. Non-cash stock compensation expense for the six months ended June 30, 2008 and June 30, 2007 was $10.4 million and $7.8 million, respectively.

As of June 30, 2008, BioMarin had cash, cash equivalents, and short-term investments totaling $575.7 million.

"Higher than expected sales of Naglazyme and Aldurazyme and the steady uptake of Kuvan drove improved financials, making this our third consecutive profitable quarter," said Jean-Jacques Bienaime, Chief Executive Officer of BioMarin. "The Kuvan launch is progressing according to plan, and we continue to be optimistic about the long-term potential of the drug, as well as the continued geographic expansion of Naglazyme, and the timely development of the PEG-PAL, BMN 110 for MPS IVA and other promising pipeline programs. In the second quarter, we licensed from Summit the preclinical candidate SMT C1100 for Duchenne muscular dystrophy, adding to our growing pipeline."

Net Product Revenue

Net product revenue from Naglazyme (galsulfase), an enzyme replacement therapy for mucopolysaccharidosis VI (MPS VI), was $35.1 million for the second quarter of 2008, an increase of 67.9 percent compared to net product revenue of $20.9 million for the second quarter of 2007. Net product revenue from Naglazyme for the six months ended June 30, 2008 was $62.8 million, an increase of 59.8 percent from net product revenue of $39.3 million for the six months ended June 30, 2007. BioMarin is commercializing Naglazyme in the United States, Europe, Latin America, and Turkey and through distributors in other international markets.

Net sales of Aldurazyme (laronidase), an enzyme replacement therapy for mucopolysaccharidosis I (MPS I) recorded by Genzyme, was $38.7 million for the second quarter of 2008, an increase of 33.0 percent compared to net sales of $29.1 million for the second quarter of 2007. Net sales of Aldurazyme recorded by Genzyme for the six months ended June 30, 2008 was $75.5 million, compared to net sales of $55.9 million for the six months ended June 30, 2007.

Net product revenue to BioMarin related to Aldurazyme for the second quarter of 2008 was $13.4 million. This reflects a reduction in net product revenue from the amount payable to BioMarin by Genzyme due to the timing of inventory transfers to Genzyme, which were less than units shipped to third party customers by Genzyme during the second quarter of 2008. Beginning January 1, 2008, as a result of the restructuring of the joint venture with Genzyme, BioMarin receives a royalty of 39.5% to 50% of worldwide net sales. In addition, BioMarin recognizes product transfer revenue when product is shipped to Genzyme. This amount will eventually be deducted from royalties earned when the product is sold by Genzyme.

Net product revenue from Kuvan (sapropterin dihydrochloride) Tablets, a product for the treatment of phenylketonuria (PKU), for the second quarter of 2008 was $12.0 million, more than doubling reported sales in the first quarter of 2008. Of the 49 U.S. patients in the extension study, approximately 73% (36 patients) have continued on therapy and transitioned to commercial therapy. Of the 36 patients who transitioned onto commercial therapy, 36 (100%) are still on Kuvan as of June 30, 2008. The majority of these patients have been on Kuvan for over two years. Also, as of June 30, 2008, of all the commercial patients on Kuvan for 90 days or more, 88% remain on therapy and are current with refills.

Collaborative Agreement Revenues

Collaborative agreement revenues for the second quarter of 2008 were $2.5 million, compared to $3.5 million for the second quarter of 2007. Collaborative agreement revenues for the six months ended June 30, 2008 were $5.0 million, compared to $7.7 million for the six months ended June 30, 2007. Collaborative agreement revenues decreased in 2008 compared to 2007 due to less reimbursable Kuvan development expenses during the second quarter and first six months of 2008.

2008 Financial Guidance

BioMarin estimates 2008 net sales of Naglazyme will be in the range of $130 million to $140 million, revised from a range of $115 million to $125 million.

Net sales of Aldurazyme by Genzyme are estimated to be in the range of $135 million to $145 million. BioMarin's net product revenue related to Aldurazyme is estimated to be between $72 million and $80 million, revised from a range of $70 million to $80 million, which includes both the royalty earned on net sales by Genzyme and incremental product revenue related to the transfer of Aldurazyme inventory to Genzyme to meet future product demand.

BioMarin estimates 2008 Kuvan net sales to be in the range of $45 million to $65 million, revised from a range of $45 million to $70 million.

BioMarin estimates its Generally Accepted Accounting Principles (GAAP) net income for the fiscal year ending December 31, 2008 will be in the range of $30 million to $42 million, revised from a range of $28 million to $40 million, which includes the impact of the recently announced licensing deal with Summit and assumes that the $30 million milestone for EU Kuvan approval will be earned in 2008. The net income estimate includes approximately $24 million to $27 million in non-cash stock compensation expense. Non-GAAP net income, excluding the impact of non-cash stock compensation, is estimated to be in the range of $54 million to $69 million, revised from a range of $52 million to $67 million.

Non-GAAP Financial Information and Reconciliation

The above 2007 and 2008 second quarter and first half results and 2008 financial guidance include actual and Management's 2008 estimated net income, respectively, determined in accordance with GAAP and non-GAAP net income. As used in this release, non-GAAP income is net income calculated in accordance with GAAP, but excluding non-cash stock compensation expense, a non-GAAP financial measure. Stock compensation expense excluded in the calculation of non-GAAP net income was $5.9 million for the second quarter of 2008, $4.3 million for the second quarter of 2007, $10.4 million for the six months ended June 30, 2008, $7.8 million for the six months ended June 30, 2007 and $24.0 million to $27.0 million for Management's estimate of 2008 net income. The reconciliation of this measure to the estimated GAAP net income is as follows (in millions):

                                                                   2008
                         Q2 2008  Q2 2007  1H 2008  1H 2007     Management
                                                                 Guidance

  GAAP net income (loss)   $3.8   ($3.9)    $5.5    ($13.2)    $30.0 - $42.0
  Non-cash stock
   compensation expense     5.9     4.3     10.4       7.8      24.0 -  27.0
  Non-GAAP net income
   (loss)                  $9.7    $0.4    $15.9     ($5.4)    $54.0 - $69.0


Management believes that this non-GAAP information is useful to investors, taken in conjunction with BioMarin's GAAP information because Management uses such information internally for its operating, budgeting and financial planning purposes, and to enhance investors' overall understanding of the company's prospects for the future.

  Anticipated Upcoming Milestones
  -- 4Q08: Results from Phase 2 sickle cell disease trial
  -- 4Q08: Results from BH4+Vitamin C study
  -- 4Q08: Kuvan approval by EMEA
  -- 4Q08/1Q09: Results from PEG-PAL Phase 1 trial
  -- 1Q09: Results from Phase 2 PAD trial
  -- 1Q09: Results from Phase 2 PAH trial
  -- 1Q09: Results from proteinuria in chronic kidney disease trial
  -- 1Q09: Initiation of PEG-PAL Phase 2 trial
  -- 1Q09: Initiation of Phase 1 trial for ERT for MPS IVA
  -- 1H09: Initiation of Phase 1 trial for Duchenne muscular dystrophy


  Upcoming Investor Conferences and Events
  -- September 9: Morgan Stanley Healthcare Conference -- New York City
  -- September 16-18: Merrill Lynch Global Healthcare Conference -- London
  -- September 22-25: UBS Global Life Sciences Conference -- New York City
  -- September 23-24: Biotech in Europe Investor Forum


BioMarin will host a conference call and webcast to discuss second quarter 2008 financial results today, Tuesday, August 5, at 5:00 p.m. ET (22:00 CET). This event can be accessed on the investor section of the BioMarin website at http://www.bmrn.com/.

  Date: August 5, 2008
  Time: 5:00 p.m. ET (22:00 CET)
  U.S. / Canada Dial-in Number:  866.700.0161
  International Dial-in Number:  617.213.8832
  Participant Code: 99466709
  Replay Dial-in Number: 888.286.8010
  Replay International Dial-in Number: 617.801.6888
  Replay Code: 94527503


  About BioMarin

BioMarin develops and commercializes innovative biopharmaceuticals for serious diseases and medical conditions. The company's product portfolio comprises three approved products and multiple clinical and pre-clinical product candidates. Approved products include Naglazyme(R) (galsulfase) for mucopolysaccharidosis VI (MPS VI), a product wholly developed and commercialized by BioMarin; Aldurazyme(R) (laronidase) for mucopolysaccharidosis I (MPS I), a product which BioMarin developed through a 50/50 joint venture with Genzyme Corporation; and Kuvan(R) (sapropterin dihydrochloride) Tablets, a product for the treatment of phenylketonuria (PKU), developed in partnership with Merck Serono, a division of Merck KGaA of Darmstadt, Germany. Other product candidates include 6R-BH4 for cardiovascular indications, which is currently in Phase 2 clinical development for the treatment of peripheral arterial disease and sickle cell disease, and PEG-PAL (PEGylated recombinant phenylalanine ammonia lyase), which is currently in Phase 1 clinical development for the treatment of PKU. For additional information, please visit http://www.bmrn.com/. Information on BioMarin's website is not incorporated by reference into this press release.

Forward-Looking Statement

This press release contains forward-looking statements about the business prospects of BioMarin Pharmaceutical Inc., including, without limitation, statements about: the expectations of revenue and sales related to Naglazyme, Kuvan, and Aldurazyme; the financial performance of the BioMarin as a whole; the timing of BioMarin's clinical trials of 6R-BH4 for other indications, PEG-PAL and other product candidates; the continued clinical development and commercialization of Aldurazyme, Naglazyme, Kuvan, and its product candidates; and actions by regulatory authorities. These forward-looking statements are predictions and involve risks and uncertainties such that actual results may differ materially from these statements. These risks and uncertainties include, among others: our success in the continued commercialization of Naglazyme and Kuvan; Genzyme Corporation's success in continuing the commercialization of Aldurazyme; results and timing of current and planned preclinical studies and clinical trials; our ability to successfully manufacture our products and product candidates; the content and timing of decisions by the U.S. Food and Drug Administration, the European Commission and other regulatory authorities concerning each of the described products and product candidates; the market for each of these products and particularly Aldurazyme, Naglazyme and Kuvan; actual sales of Aldurazyme, Naglazyme and Kuvan; Merck Serono's activities related to Kuvan; and those factors detailed in BioMarin's filings with the Securities and Exchange Commission, including, without limitation, the factors contained under the caption "Risk Factors" in BioMarin's 2007 Annual Report on Form 10-K, and the factors contained in BioMarin's reports on Form 10-Q. Stockholders are urged not to place undue reliance on forward-looking statements, which speak only as of the date hereof. BioMarin is under no obligation, and expressly disclaims any obligation to update or alter any forward-looking statement, whether as a result of new information, future events or otherwise.

BioMarin(R) , Naglazyme(R) and Kuvan(R) are a registered trademarks of BioMarin Pharmaceutical Inc.

  Aldurazyme(R) is a registered trademark of BioMarin/Genzyme LLC.



              BIOMARIN PHARMACEUTICAL INC. AND SUBSIDIARIES

                       CONSOLIDATED BALANCE SHEETS
           (In thousands, except for share and per share data)

                                             December 31,       June 30,
                                               2007 (1)           2008
                                                               (unaudited)
                 ASSETS
  Current assets:
     Cash and cash equivalents                 $228,343         $252,209
     Short-term investments                     357,251          323,448
     Accounts receivable, net                    16,976           52,156
     Advances to BioMarin/Genzyme LLC             2,087              248
     Inventory                                   32,445           61,802
     Other current assets                         7,195           11,951
        Total current assets                    644,297          701,814
  Investment in BioMarin/Genzyme LLC             44,881              315
  Property, plant and equipment, net             76,818          105,199
  Intangible assets, net                          9,596            7,244
  Goodwill                                       21,262           21,262
  Restricted cash                                 2,889            5,008
  Other assets                                   15,536           13,713
        Total assets                           $815,279         $854,555

                 LIABILITIES AND STOCKHOLDERS' EQUITY
  Current liabilities:
     Accounts payable and accrued liabilities   $49,907          $54,267
     Current portion of acquisition
      obligation, net of discount                 6,309            5,816
     Deferred revenue                             5,327            3,236
     Other current liabilities                        -              104
        Total current liabilities                61,543           63,423
  Convertible debt                              497,375          497,245
  Long-term portion of acquisition
   obligation, net of discount                   66,553           65,752
  Other long-term liabilities                     2,082            2,108
        Total liabilities                       627,553          628,528
  Stockholders' equity:
     Common stock, $0.001 par value:
      250,000,000 shares authorized at
      December 31, 2007 and June 30, 2008;
      97,114,159 and 99,208,948 shares
      issued and outstanding at December 31,
      2007 and June 30, 2008, respectively           97               99
     Additional paid-in capital                 794,917          828,434
     Accumulated other comprehensive
      income (loss)                                 139             (575)
     Accumulated deficit                       (607,427)        (601,931)
        Total stockholders' equity              187,726          226,027
        Total liabilities and stockholders'
         equity                                $815,279         $854,555

  (1) December 31, 2007 balances were derived from the audited consolidated
      financials.



              BIOMARIN PHARMACEUTICAL INC. AND SUBSIDIARIES

                  CONSOLIDATED STATEMENTS OF OPERATIONS
        For the Three and Six Months Ended June 30, 2007 and 2008
           (In thousands, except for per share data, unaudited)

                                    Three Months Ended     Six Months Ended
                                          June 30,             June 30,
                                      2007       2008       2007     2008
  Revenues:
     Net product revenues           $20,941    $60,458    $39,276  $118,083
     Collaborative agreement
      revenues                        3,505      2,509      7,652     4,975
     Royalty and license revenues     4,438      1,207      4,795     1,513
        Total revenues               28,884     64,174     51,723   124,571
  Operating expenses:
     Cost of sales (1)                4,557      9,593      8,674    26,781
     Research and development (1)    19,186     23,755     37,345    41,383
     Selling, general and
      administrative (1)             17,295     25,203     33,555    48,872
     Amortization of acquired
      intangible assets               1,093      1,093      2,185     2,185
        Total operating expenses     42,131     59,644     81,759   119,221
  Income (loss) from operations     (13,247)     4,530    (30,036)    5,350
  Equity in the income (loss) of
   BioMarin/Genzyme LLC               6,550       (587)    12,713    (1,120)
  Interest income                     6,907      4,101     10,601     9,750
  Interest expense                   (3,720)    (4,081)    (6,055)   (8,193)
  Income (loss) before income
   taxes                             (3,510)     3,963    (12,777)    5,787
  Provision for income taxes            354        153        380       291
     Net income (loss)              $(3,864)    $3,810   $(13,157)   $5,496
     Net income (loss) per share,
      basic                           (0.04)     $0.04     $(0.14)    $0.06
     Net income (loss) per share,
      diluted                        $(0.04)     $0.04     $(0.14)    $0.05
  Weighted average common shares
   outstanding, basic                95,796     98,923     95,180    98,285
  Weighted average common shares
   outstanding, diluted              95,796    104,120     95,180   103,948

  (1) The following is the stock-based compensation expense included in the
      respective captions of the condensed consolidated statements of
      operations above:

                                      Three Months Ended   Six Months Ended
                                           June 30,             June 30,
                                        2007      2008        2007     2008
  Stock-based compensation expense:
     Cost of goods sold                 $117       $393       $281      $590
     Research and development          1,554      2,059      2,903     3,616
     Selling, general and
      administrative                   2,583      3,496      4,634     6,206
  Total stock-based compensation
   expense, net of tax                $4,254     $5,948     $7,818   $10,412



   Contact:

   Investors                          Media
   Eugenia Shen                       Susan Berg
   BioMarin Pharmaceutical Inc.       BioMarin Pharmaceutical Inc.
   (415) 506-6570                     (415) 506-6594

First Call Analyst:
FCMN Contact: eshen@bmrn.com

SOURCE: BioMarin Pharmaceutical Inc.

CONTACT: Investors, Eugenia Shen, +1-415-506-6570, or Media, Susan Berg,
+1-415-506-6594, both of BioMarin Pharmaceutical Inc.


BioMarin

Diseases

Lysosomal Storage Disorders (MPS I, MPS IVA, MPS VI, CLN2 disease) PKU

Products

Palynziq®
Brineura®
Vimizim®
Kuvan®
Naglazyme®
Aldurazyme®

Patient/Physician Support Contact Information

BioMarin RareConnections
Tel: 866.906.6100
Fax: 888.863.3361
E-mail: support@biomarin-rareconnections.com

Global Medical Information

Contact Global Medical Information