Change to Financial Calendar for 2023 and update regarding class action lawsuit in United States

Company announcement

Orphazyme A/SNo. 01/2023 Ole Maaløes Vej 3DK-2200 Copenhagen Nwww.orphazyme.com

Company Registration No. 32266355

Copenhagen, Denmark, April 17, 2023 – Orphazyme A/S (ORPHA.CO) (“Orphazyme” or the “Company”), announces that publication of its financial results for the full year ending December 31, 2022, previously expected April 19, 2023, are now expected to be published on April 25, 2023. Additional time is required to conclude the presentation of the financial statements. The updated financial calendar for 2023 is available on the Company’s website.

The Company also announces that the parties to the previously communicated class action lawsuit filed in the United States in July 2021 have reached an agreement in principle to settle the lawsuit in its entirety. The parties are now undertaking to prepare mutually agreeable settlement documents, and the Court has stayed all proceedings in the lawsuit pending the filing of the parties’ settlement documents. The parties currently anticipate that a motion seeking the Court’s preliminary approval of the settlement will be filed in May 2023 and that, if the Court grants preliminary approval, final court approval will be sought after members of the proposed settlement class have been afforded an opportunity to object to or opt out of the settlement. The Company will provide an update when there has been final Court judgment with respect to the settlement.

For additional information, please contact

Orphazyme A/S

Anders Vadsholt, Chief Executive Officer and Chief Financial Officer +45 2898 9055

Forward-looking statement This company announcement includes “forward-looking statements” within the meaning of the “safe harbor” provisions of the U.S. Private Securities Litigation Reform Act of 1995, including, without limitation, statements about the expected timing for the Company’s annual report. The words “expect” and “anticipate” and variations of these words or similar words are intended to identifying forward-looking statements. Although the Company believes its expectations are based on reasonable assumptions, all statements other than statements of historical fact included in this company announcement about future events are subject to (i) change without notice and (ii) factors beyond the Company’s control, including pursuant to regulatory or judicial intervention. Except as required by law, the Company assumes no obligation to update these forward-looking statements publicly, or to update the reasons actual results could differ materially from those anticipated in the forward-looking statements, even if new information becomes available in the future.

Attachment