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BioMarin Announces First Quarter 2004 Financial Results
Conference Call and Webcast to be Held Today at 12:00 p.m. EDT (18:00 CEST)
May 3, 2004
NOVATO, Calif.

BioMarin Pharmaceutical Inc. today announced financial results for the first quarter of the fiscal year 2004. The company incurred a net loss of $19.9 million ($0.31 per share) for the quarter ended March 31, 2004 compared to $19.7 million ($0.35 per share) for the quarter ended March 31, 2003.

As of March 31, 2004, BioMarin had cash, cash equivalents, and short-term investments of approximately $191.2 million.

The net loss in the first quarter of 2004 was due primarily to: the continued clinical development of Phase 3 product candidate Aryplase(TM) for the treatment of mucopolysaccharidosis VI (MPS VI); BioMarin's share of the loss from BioMarin/Genzyme LLC related to Aldurazyme(R); and expenses related to the advancement of the company's phenylketonuria (PKU) clinical development program.

Fredric D. Price, Chairman and Chief Executive Officer of BioMarin stated, "We are pleased with the progress we have made this year in our transformation to a fully integrated, pediatric-focused biopharmaceutical company with commercial operations in the U.S. We anticipate completion of the recently announced Ascent Pediatrics transaction soon, and in this current quarter we will announce the results of the just completed Phase 3 trial of Aryplase for MPS VI. If the results from that trial are positive, we anticipate filing applications for approval of Aryplase in the U.S. and the E.U. in the fourth quarter of 2004."

  First Quarter Highlights
  Ascent Pediatrics Transaction

  -- On April 20, 2004, BioMarin announced the signing of a definitive
     agreement with Medicis Pharmaceutical Corporation to obtain its
     pediatric business, Ascent Pediatrics, Inc.  The transaction, which is
     expected to close in the second quarter of 2004, brings with it
     Orapred(R), a U.S. Food and Drug Administration (FDA) approved,
     patent-protected drug to treat asthma in children, two additional
     proprietary formulations of Orapred, and a U.S. pediatric-focused sales
  -- Excluding the non-cash charges associated with the transaction,
     BioMarin expects that the net loss for 2004 will be less than the
     previously announced guidance of a loss between $68 million to
     $70 million. The company expects that the net sales of Orapred will be
     approximately $42 million for the 12 months ending December 31, 2004.
     BioMarin will record all Orapred revenues beginning with the
     transaction closing.
  -- BioMarin expects the loss for 2005 will decrease significantly due to
     the anticipated profits from Orapred and Aldurazyme, an enzyme
     replacement therapy for the treatment of mucopolysaccharidosis I
     (MPS I), marketed on a worldwide basis by Genzyme Corporation.
     BioMarin will provide more specific revised guidance for 2004 and 2005
     during the third quarter of this year and at the end of this year,

  Aldurazyme for MPS I
  -- Sales of Aldurazyme by BioMarin/Genzyme LLC were $7.4 million for the
     first quarter of 2004.  Previously announced sales guidance for the
     12 months ending December 31, 2004, provided by Genzyme Corporation,
     remains unchanged at between $40 million and $44 million.  Aldurazyme
     received marketing approval from the FDA and the European Commission in
     the second quarter of 2003 and since then, Aldurazyme has been approved
     in Norway, Iceland, Israel, and the Czech Republic, with additional
     applications pending in Canada, Australia, New Zealand, Russia, Korea,
     and Bulgaria.

  Aryplase for MPS VI
  -- BioMarin recently completed the Phase 3 trial of Aryplase for MPS VI
     and expects to announce the results in the second quarter of 2004,
     following analysis of the data from the six clinical sites.  If the
     data are positive, the company plans to file simultaneous applications
     for approval in the U.S. and European Union in the fourth quarter of

  Phenoptin(TM) for PKU
  -- On February 23, 2004, BioMarin announced the initiation of its PKU
     clinical program beginning with a pilot trial of tetrahydrobiopterin
     (6R-BH4).  The company expects to announce data from this trial in the
     third quarter and, pending positive trial results, to initiate an
     additional clinical trial in late 2004.

  Upcoming Presentations at Medical Conferences

Investigators will present data from ongoing studies of Aldurazyme for MPS I and Aryplase for MPS VI at the following three conferences:

3rd International Conference: Prospects in the Treatment of Rare Diseases, May 19-22, 2004, Trieste, Italy

  Aryplase/ MPS VI
  -- Enzyme Replacement Therapy for Skeletal Disease in
     Mucopolysaccharidosis Type VI

  Immune Tolerance
  -- Prospects for Treating Null-Genotyped Patients with Neurologic Disease
     Using Immune Tolerance and Intrathecal Enzyme Therapy

8th International Symposium on MPS and Related Diseases, June 10 - 13, 2004, Mainz, Germany

  Aldurazyme/ MPS I
  -- Aldurazyme(R) (laronidase) Enzyme Replacement Therapy in MPS I:
     Preliminary Data in Children Less Than Five Years of Age
  -- The Attenuated ('Non-Hurler') Phenotype of Mucopolysaccharidosis: A
     Review of the Clinical Features in 29 Patients
  -- MPS I Characteristics: Description of First Patients Entered into the
     MPS I Registry
  -- Update on Clinical Trial Results in MPS I
  -- The MPS I Registry: Centralised Data Collection to Outline the Natural
     History and Treatment of MPS I Disease
  -- Successful Treatment of the Brain and Meninges with Immune Tolerance in
     Canine MPS I

  Aryplase/ MPS VI
  -- Update on Phase 1, 2, and 3 Clinical Trial Results of Aryplase in MPS
  -- A Threshold Effect of Urinary Glycosaminoglycans and the Walk Test as
     Indicators of Disease Progression in a Survey of Subjects with
     Mucopolysaccharidosis VI (Maroteaux-Lamy Syndrome)
  -- Long-Term Combined Therapy with Recombinant Human
     N-acetylgalactosamine-4-Sulfatase for Degenerative Joint Disease in
     Mucopolysaccharidosis VI Cats
  -- Flexible Endoscopy in a 13-Year-Old Boy with MPS VI and Tracheostomy
     Because of Upper Airway Obstruction: Changes After 91 and 146 Weeks of
     a Phase I/II Enzyme Replacement Therapy with Recombinant Human
     Arylsulfatase B

36th European Human Genetics Conference, June 12 - 15, 2004, Munich, Germany

  Aryplase/ MPS VI
  -- Update on Phase 1, 2, and 3 Clinical Trial Results of Aryplase in MPS
  -- A Threshold Effect of Urinary Glycosaminoglycans and the Walk Test as
     Indicators of Disease Progression in a Survey of Subjects with
     Mucopolysaccharidosis VI (Maroteaux-Lamy Syndrome)

  Upcoming Corporate Presentations

BioMarin will present an overview of its business and product development programs at the following upcoming healthcare and biotechnology investment conferences:

  -- Rodman & Renshaw Global Healthcare Conference, May 12-14, 2004, London,
     United Kingdom
  -- BioEquity Europe 2004, May 17-18, 2004, Edinburgh, United Kingdom
  -- UBS 2004 Global Specialty Pharmaceutical Conference, May 24-25, 2004,
     New York City, NY
  -- Pacific Growth Equities Life Sciences Growth Conference, June 9-10,
     2004, San Francisco, CA
  -- Needham & Co. Third Annual Biotechnology Conference, June 16-17, 2004,
     New York City, NY

BioMarin will host a conference call and webcast to discuss first quarter financial results today at 12:00 p.m. EDT (18:00 CEST). This event can be accessed on the BioMarin website at: http://investor.biomarinpharm.com/ .

   Date:  May 4, 2004
   Time:  12:00 p.m. EDT (18:00 CEST)
   U.S. & Canada Toll-free Dial in #:  1-800-915-4836
   International Dial in #:  973-317-5319
   Replay Toll-free Dial in #:  1-800-428-6051
   Replay International Dial in #:  973-709-2089
   Replay Code #:  346222

BioMarin Pharmaceutical Inc. develops innovative biopharmaceuticals and commercializes therapeutics for serious pediatric diseases.

Forward-Looking Statement

This press release contains forward-looking statements about the business prospects of BioMarin Pharmaceutical Inc., including, without limitation, statements about: the possible acquisition of the Ascent Pediatrics, Inc. business and the financial performance of that business if the transaction is completed; the financial performance of Aldurazyme and the company as a whole; the continued development and commercialization of Aldurazyme, Aryplase and Phenoptin; 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: actual completion of the proposed transaction; the successful integration of the sales force and the Orapred business into BioMarin's organization; results and timing of current and planned preclinical studies and clinical trials; the content and timing of decisions by the FDA, the European Commission and other regulatory authorities concerning each of the described products; the market for each of these products and particularly Aldurazyme and Orapred; actual sales of Aldurazyme and Orapred; the possible development of competing products; and those factors detailed in BioMarin's filings with the Securities and Exchange Commission, including, without limitation, the factors contained under the caption "Factors That May Affect Future Results" in BioMarin's 2003 Annual Report on Form 10-K and the factors contained in BioMarin's reports on Forms 10-Q and 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.

BioMarin's press releases and other company information are available online at www.BMRN.com. Information on BioMarin's website is not incorporated by reference into this press release.

NOTE: Aldurazyme(R) is a registered trademark of BioMarin/Genzyme LLC. All rights reserved.

Orapred(R) is a registered trademark of Ascent Pediatrics, Inc., a wholly owned subsidiary of Medicis Pharmaceutical Corporation.

CONTACT: Joshua A. Grass, Sr. Manager, Investor and Financial Relations, +1-415-506-6777, or Susan Ferris Manager, Corporate Communications, +1-415-506-6701, both of BioMarin Pharmaceutical Inc.

              BioMarin Pharmaceutical Inc. and Subsidiaries
                       Consolidated Balance Sheets
             (In thousands, except share and per share data)

                                                December 31,     March 31,
                                                   2003 (1)         2004
  Current assets:
     Cash and cash equivalents                    $121,406        $96,199
     Short-term investments                         84,951         94,999
     Investment in and advances to
      BioMarin/Genzyme LLC                          16,058         15,149
     Other current assets                            2,854          2,478

       Total current assets                        225,269        208,825

  Property and equipment, net                       25,154         24,447
  Other assets                                       5,917          6,024

       Total assets                               $256,340       $239,296

  Liabilities and Stockholders' Equity
  Current liabilities:
     Accounts payable and accrued liabilities      $10,098        $12,850
     Other current liabilities                       2,717          2,311

       Total current liabilities                    12,815         15,161

  Convertible debt                                 125,000        125,000
  Other long-term liabilities                          672            363

       Total liabilities                           138,487        140,524

  Stockholders' equity:
     Common stock, $0.001 par value:
      150,000,000 shares authorized;
      64,156,285 and 64,273,601 shares issued
      and outstanding December 31, 2003 and
      March 31, 2004, respectively                      64             64
     Additional paid-in capital                    414,110        414,929
     Warrants                                        5,219          5,219
     Deferred compensation                           (145)          (101)
     Accumulated other comprehensive loss             (17)           (16)
     Accumulated deficit                         (301,378)      (321,323)

       Total stockholders' equity                  117,853         98,772

       Total liabilities and stockholders'
        equity                                    $256,340       $239,296

  (1) December 31, 2003 balances were derived from the audited consolidated
      financial statements.

              BioMarin Pharmaceutical Inc. and Subsidiaries
                  Consolidated Statements of Operations
            For the Three Months Ended March 31, 2003 and 2004
             (In thousands, except per share data, unaudited)

                                                      Three Months Ended
                                                          March 31,
                                                     2003           2004

  Operating expenses:
     Research and development                      $10,991        $13,887
     General and administrative                      2,799          3,689
     Equity in the loss of BioMarin/Genzyme LLC      6,753          1,759

       Total operating expenses                     20,543         19,335

  Loss from operations                            (20,543)       (19,335)
  Interest income                                      413            761
  Interest expense                                   (130)        (1,371)

  Net loss from continuing operations             (20,260)       (19,945)
  Gain on disposal of discontinued operations          577             --

      Net loss                                   $(19,683)      $(19,945)

  Net loss per share, basic and diluted:
      Net loss from continuing operations          $(0.36)        $(0.31)
      Gain on disposal of discontinued operations     0.01             --

      Net loss                                     $(0.35)        $(0.31)

  Weighted average common shares outstanding        56,964         64,225

SOURCE: BioMarin Pharmaceutical Inc.

CONTACT: Joshua A. Grass, Sr. Manager, Investor and Financial Relations,
+1-415-506-6777, or Susan Ferris Manager, Corporate Communications,
+1-415-506-6701, both of BioMarin Pharmaceutical Inc.