To date, and to our knowledge, no case of severe hemophilia A patients receiving long-term anticoagulation has been published. Severe hemophilia A is a hereditary bleeding disorder characterized by a factor VIII (FVIII) deficiency of \<1%. Anticoagulation remains a real challenge in these patients, given the precarious hemostatic balance between the bleeding risk associated with anticoagulation and the antithrombotic protection associated with factor VIII deficiency. The advent of new replacement therapies, characterized by FVIII molecules with a prolonged or very prolonged half-life, provides a high level of FVIII coverage (and therefore protection against the risk of bleeding) in patients receiving prophylaxis, thus facilitating the initiation of anticoagulation therapy.