Background: Symptomatic hemophilia A is a rare disorder in females. Pregnancy and delivery in such women can be life threatening. Obstetric management is challenging and requires a multidisciplinary approach to ensure a good outcome.
Case: A woman with hemophilia A delivered by cesarean developed a deep vein thrombosis 10 days postpartum after recombinant factor VIII administration.
Conclusion: Hemophilia A due to skewed X-inactivation is a rare cause of peripartum bleeding. Recombinant factor VIII administration can prevent hemorrhage during and after cesarean delivery but may be associated with development of deep vein thrombosis.