BioMarin Pharmaceutical Inc.

Item 1.01 Entry into a Material Definitive Agreement. On September 24, 2026, BioMarin Pharmaceutical Inc. (BioMarin) entered into a Settlement and License Agreement (the Agreement) with Ascendis Pharma A/S (Ascendis). The terms of the Agreement are consistent with those contained in the binding term sheet previously entered into by the parties, which was reported by BioMarin on a Current Report on Form 8-K filed with the Securities and Exchange Commission on August 31, 2026 (the Term Sheet). The Agreement supersedes and replaces the Term Sheet and is deemed effective as of August 28, 2026 (the Effective Date). In accordance with the Agreement and consistent with the previously reported terms and conditions of the Term Sheet, BioMarin has granted to Ascendis and its affiliates a non-exclusive, worldwide, transferable, royalty-bearing license to certain patents (the BioMarin Patent Rights) to research, develop, manufacture, use, sell, and commercialize any product, method, or service that includes the C-type natriuretic peptide known as “TransCon CNP,” “navepegritide,” or “Yuviwel” (collectively, the Licensed Products) for all current and potential indications, including achondroplasia and hypochondroplasia, and in combination with other medicines. In consideration for the rights and licenses granted to Ascendis, Ascendis will pay to BioMarin a royalty equal to 20% of annual net sales of Licensed Products in the United States and 18% of annual net sales of Licensed Products in the European Union, Brazil and South Korea, in each case, from the date of the first commercial sale in each territory (applied retroactively, as applicable) until May 2030. Pursuant to the Agreement, the parties will also dismiss with prejudice all pending litigation between them relating to the BioMarin Patent Rights. The Agreement also contains a mutual regulatory non-interference clause, a covenant by Ascendis to not challenge BioMarin’s Patent Rights, a release by BioMarin of claims against Ascendis for infringement of BioMarin’s Patent Rights prior to the Effective Date and a covenant not to sue Ascendis for infringement of BioMarin’s Patent Rights, and certain other terms that are customary for transactions of this nature. Forward-Looking Statements This Current Report on Form 8-K contains forward-looking statements about the entry into the Agreement with Ascendis and the transactions contemplated therein, including, without limitation, statements about: the expected benefits of such transactions, including anticipated royalty payments; expectations regarding future commercialization of Licensed Products; and plans and expectations regarding the dismissal of the pending legal proceedings. 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: BioMarin’s ability to enforce the Term Sheet or the Agreement; actual sales of Licensed Products; 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 Quarterly Report on Form 10-Q for the quarter ended June 30, 2026, as such factors may be updated by any subsequent reports. Investors 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.