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BioMarin Announces Third Quarter 2006 Financial Results
Robust Naglazyme Launch Drives Better Bottom-Line Results
Conference Call and Webcast to Be Held Today at 5:00 p.m. ET (23:00 CET)
Nov 1, 2006
PRNewswire-FirstCall
NOVATO, Calif.

BioMarin Pharmaceutical Inc. (Nasdaq and SWX: BMRN) today announced results for its third quarter ended September 30, 2006. The net loss was $7.0 million ($0.08 per share) for the third quarter of 2006, compared to $15.5 million ($0.21 per share) for the third quarter of 2005. The third quarter results include $3.3 million in expenses ($0.04 per share) incurred in connection with the partial conversion of convertible notes due 2008 into shares of common stock. The net loss was $18.1 million ($0.22 per share) for the nine months ended September 30, 2006, compared to $59.3 million ($0.88 per share) for the nine months ended September 30, 2005, representing a reduction of $41.2 million, or approximately 69 percent.

Jean-Jacques Bienaime, Chief Executive Officer of BioMarin commented, "The launch of Naglazyme in the U.S., Europe and some international markets continues to exceed our expectations. Consequently, we expect 2006 net product sales of Naglazyme to be slightly higher and our net loss for 2006 to be lower than we had previously projected."

Mr. Bienaime continued, "Our recent discussions with the FDA related to Phenoptin development in PKU indicate that we remain on track for a potential U.S. approval of Phenoptin in late 2007, assuming a favorable review of the NDA and assuming that we receive priority review. The FDA indicated that we have enough clinical data to file the NDA as planned. Although not required by the FDA, we have decided to include data from the diet study, which could support a younger age range on the label at launch. As such, we have adjusted our projected Phenoptin NDA filing date from late Q107 to Q207. With regard to our cardiovascular program, 116 patients were quickly enrolled in the CONTROL study of 6R-BH4 in poorly controlled hypertension, well beyond our initial target of 80, and we expect to announce results from the study in the first quarter of 2007. We are also on track to initiate a study of 6R-BH4 in peripheral arterial disease by the end of the year."

Product Sales

Net sales of Naglazyme(R) (galsulfase), an enzyme replacement therapy for mucopolysaccharidosis VI (MPS VI), were $12.9 million for the third quarter of 2006, compared to $10.3 million for the second quarter of 2006, representing a sequential increase of approximately 25 percent. Naglazyme net sales were $30.2 million for the nine months ended September 30, 2006. Naglazyme was approved by the U.S. Food and Drug Administration (FDA) in late May 2005, and by the European Commission in late January 2006. Naglazyme net sales for the three and nine months ended September 30, 2005 were $2.3 million and $2.4 million, respectively. BioMarin is commercializing Naglazyme in the United States, Europe, Latin America, and through distributors in other international markets.

Net sales of Aldurazyme(R) (laronidase), an enzyme replacement therapy for mucopolysaccharidosis I, (MPS I) by BioMarin/Genzyme LLC increased by approximately 24 percent to $25.0 million for the third quarter of 2006, compared to $20.1 million in the third quarter of 2005. Net sales for the nine months ended September 30, 2006 increased by approximately 27 percent to $69.9 million, compared to $55.2 million for the nine months ended September 30, 2005. BioMarin's share of the profit of BioMarin/Genzyme LLC was $5.1 million for the third quarter of 2006, compared to a profit of $3.4 million for the third quarter of 2005. BioMarin's share of the profit from BioMarin/Genzyme LLC for the nine months ended September 30, 2006 was $13.6 million, compared to $8.8 million for the nine months ended September 30, 2005.

Royalty and License Revenues

Royalty and license revenues for the third quarter and nine months of 2006 were $5.4 million and $15.0 million, respectively, and include royalties on net product sales of the Orapred product line, including Orapred(R) (prednisolone sodium phosphate oral solution) and Orapred ODT(TM) (prednisolone sodium phosphate orally disintegrating tablets). BioMarin will also receive an additional milestone payment of $4.0 million on the first anniversary of FDA approval of Orapred ODT in June 2007.

As of September 30, 2006 BioMarin had cash, cash equivalents and short-term investments totaling $293.9 million.

  Financial Guidance

  2006 Projected Net Product Sales

BioMarin has updated its net sales guidance for Naglazyme for the fiscal year ending December 31, 2006. BioMarin estimates 2006 net sales of Naglazyme to be in the range of $43 million to $45 million, compared to the previously estimated range of $40 million to $44 million.

BioMarin reconfirms estimated sales of Aldurazyme through the joint venture for 2006 to be in a range of $90 million to $100 million.

2006 Projected Net Loss

BioMarin has updated its estimated GAAP net loss for the fiscal year ending December 31, 2006 from a range of $33 million to $37 million to a range of $28 million to $32 million, which includes $8.4 million of expenses related to the 2004 acquisition of Orapred and approximately $9.0 million of stock compensation expense.

  Recent Events and Third Quarter 2006 Highlights
  -- On October 31, BioMarin announced marketing approval for Aldurazyme in
     Japan.

  -- On September 25, BioMarin announced the completion of an exchange of a
     portion of its 3.5% convertible subordinated notes due 2008 for common
     stock.  The remaining value of the notes outstanding is $51.4 million.

  -- On September 20, BioMarin announced that it completed enrollment of the
     Phase 2 clinical study of 6R-BH4 for the treatment of poorly controlled
     hypertension (also known as the CONTROL study).  The company remains on
     track to announce data from this study in the first quarter of 2007.

  -- On September 12, BioMarin and Serono announced data from clinical
     studies of Phenoptin(TM) (sapropterin dihydrochloride), an
     investigational oral small-molecule therapeutic for the treatment of
     phenylketonuria (PKU), that were presented at the 56th Annual Meeting
     of the American Society of Human Genetics in New Orleans, Louisiana,
     held October 9 to 13, 2006.

BioMarin will host a conference call and webcast to discuss third quarter financial results today, Wednesday, November 1, at 5:00 p.m. ET (23:00 CET). This event can be accessed on the investor section of the BioMarin website at www.BMRN.com.

  Date:  November 1, 2006
  Time:  5:00 p.m. ET (23:00 CET)
  U.S. & Canada Toll-free Dial in #:  800.299.7089
  International Dial in #:  617.801.9714
  Participant Code:  17913042
  Replay Toll-free Dial in #:  888.286.8010
  Replay International Dial in #:  617.801.6888
  Replay Code:  74682255

  About BioMarin

BioMarin develops and commercializes innovative biopharmaceuticals for serious diseases and medical conditions. The company's product portfolio is comprised of two approved products and multiple clinical and preclinical product candidates. Approved products include Naglazyme(R) (galsulfase) for mucopolysaccharidosis VI (MPS VI), a product wholly developed and commercialized by BioMarin, and Aldurazyme(R) (laronidase) for mucopolysaccharidosis I (MPS I), a product which BioMarin developed through a 50/50 joint venture with Genzyme Corporation. Investigational product candidates include Phenoptin(TM) (sapropterin dihydrochloride), a Phase 3 product candidate for the treatment of phenylketonuria (PKU), and 6R-BH4 for cardiovascular indications, which is currently in Phase 2 clinical development for the treatment of poorly controlled hypertension. For additional information, please visit 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 sales expectations of BioMarin's product Naglazyme and BioMarin/Genzyme LLC's product Aldurazyme; Alliant Pharmaceuticals' commercialization of Orapred ODT; the financial performance of the BioMarin as a whole; the timing of BioMarin's clinical trials of Phenoptin and 6R-BH4 for other indications; the continued clinical development and commercialization of Aldurazyme, Naglazyme, Phenoptin, and 6R-BH4 for other indications; and actions by regulatory authorities, including actions related to Naglazyme, Phenoptin, and 6R-BH4 for other indications. 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 continuing commercialization of Naglazyme; our joint venture partner's success in continuing the commercialization of Aldurazyme; Alliant Pharmaceuticals' success in commercializing Orapred ODT; 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 Orapred ODT; actual sales of Aldurazyme, Naglazyme and Orapred ODT; 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 2005 Annual Report on Form 10-K, as amended, and the factors contained in BioMarin's reports on Form 8-K. 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.

NOTE: Naglazyme(R) is a registered trademark of BioMarin Pharmaceutical Inc.

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

Orapred(R) is a registered trademark of Medicis Pediatrics, Inc. and is used under license.

  Contact:
  Investors and Media
  Eugenia Shen
  BioMarin Pharmaceutical Inc.
  415-506-6570


Net Product Sales of BioMarin Pharmaceutical Inc. and BioMarin/Genzyme LLC
     For the Three and Nine Months Ended September 30, 2005 and 2006
                         (In millions, unaudited)


                            Three Months Ended        Nine Months Ended
                              September 30,              September 30,
                              2005      2006           2005         2006
 Aldurazyme (as
  reported
  by BioMarin/Genzyme
  LLC) (1)                 $20.1        $25.0        $55.2        $69.9
  Naglazyme                  2.3         12.9          2.4         30.2
  Orapred                    0.2          1.8          6.5          3.1

   (1) The Company recognizes its 50% share of the net income of
   BioMarin/Genzyme LLC as Equity in the income of BioMarin/Genzyme LLC in
   the Company's consolidated statements of operations.


              BIOMARIN PHARMACEUTICAL INC. AND SUBSIDIARIES

                  CONSOLIDATED STATEMENTS OF OPERATIONS
     For the Three and Nine Months Ended, September 30, 2005 and 2006
           (In thousands, except for per share data, unaudited)

                                  Three Months Ended       Nine Months Ended
                                    September 30,            September 30,
                                   2005        2006         2005       2006
  Revenues:
    Net product sales             $2,456     $14,660       $8,881   $33,297
    Collaborative agreement
     revenues                      5,123       4,908        7,313    13,857
    Royalty and license revenues      --       5,359           --    15,036
       Total revenues              7,579      24,927       16,194    62,190
  Operating expenses:
    Cost of sales (excludes
     amortization of
     developed product
     technology)                     164       2,612        1,301     5,124
    Research and development      13,894      18,105       43,708    46,163
    Selling, general and
     administrative                9,797      12,292       30,480    35,059
    Amortization of acquired
     intangible assets               286       1,093          858     2,558
       Total operating expenses   24,141      34,102       76,347    88,904
  Equity in the income of
   BioMarin/Genzyme LLC            3,388       5,059        8,766    13,604
  Loss from operations           (13,174)     (4,116)     (51,387)  (13,110)
  Interest income                    561       4,003        1,177     8,738
  Interest expense                (2,863)     (3,608)      (9,064)  (10,455)
  Debt conversion expense             --      (3,315)          --    (3,315)
      Net loss                  $(15,476)    $(7,036)    $(59,274) $(18,142)
      Net loss per share,
       basic and diluted          $(0.21)      $(0.08)     $(0.88)   $(0.22)
  Weighted average common
   shares outstanding, basic
   and diluted                    71,996       86,269      67,047    82,232


              BIOMARIN PHARMACEUTICAL INC. AND SUBSIDIARIES

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

                                           December 31,       September 30,
                                             2005 (1)             2006
                                                               (unaudited)
                        ASSETS
  Current assets:
    Cash and cash equivalents               $38,092             $191,782
    Short-term investments                    9,700              102,118
    Accounts receivable, net                  5,860               13,000
    Advances to BioMarin/Genzyme LLC          1,071                1,561
    Inventory                                10,898               25,762
    Other current assets                      3,320                5,143
      Total current assets                   68,941              339,366
  Cash balances related to long-term debt    17,049                   --
  Investment in BioMarin/Genzyme LLC         31,983               33,587
  Property, plant and equipment, net         37,321               52,469
  Acquired intangible assets, net            15,306               12,748
  Goodwill                                   21,262               21,262
  Other assets                                3,441                7,640
      Total assets                         $195,303             $467,072

          LIABILITIES AND STOCKHOLDERS' EQUITY (DEFICIT)
  Current liabilities:
    Accounts payable and
     accrued liabilities                    $20,934              $29,200
    Current portion of
     acquisition obligation,
     net of discount                         7,477                 6,787
    Current portion of
     deferred revenue                        8,096                 7,242
    Current portion of
     equipment and facility loans            3,860                    --

      Total current liabilities             40,367                43,229
  Convertible debt                         125,000               223,940
  Long-term portion of
   acquisition obligation, net
   of discount                              70,873                69,144
  Deferred revenue, net of current
   portion                                  11,825                6,796

  Equipment and facility
   loan, net of current portion             17,049                   --

  Other long-term liabilities                7,651                1,846
      Total liabilities                    272,765              344,955
  Stockholders' equity (deficit):
    Common stock, $0.001 par
     value:  150,000,000 shares
     authorized; 74,301,610 and
     91,291,305 shares issued
     and outstanding at
     December 31, 2005 and
     September 30, 2006, respectively           75                   91
    Additional paid-in capital             485,570              703,283
    Accumulated other comprehensive loss       (16)                 (24)
    Accumulated deficit                   (563,091)            (581,233)
      Total stockholders'
       equity (deficit)                    (77,462)             122,117
      Total liabilities
       and stockholders'
       equity (deficit)                   $195,303             $467,072

  (1) December 31, 2005 balances were derived from the audited consolidated
      financial statements.

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

SOURCE: BioMarin Pharmaceutical Inc.

CONTACT: Eugenia Shen of BioMarin Pharmaceutical Inc., +1-415-506-6570