Abstract Acquired hemophilia A is a rare but potentially life‐threatening hemorrhagic disorder caused by the development of autoantibodies directed mostly against coagulation factor VIII. Acquired hemophilia is frequently associated with several underlying conditions such as malignancy, autoimmune disorders, drug reactions, and pregnancy, although the pathogenesis remains undetermined (idiopathic) in up to 50% of reported cases. The disorder occurs most commonly in the elderly and only rarely affects pediatric patients, who might however experience severe, and sometimes life‐threatening, hemorrhage. The maternal transplacental transfer of the autoantibody to the neonate occurs very rarely and also in this circumstance it may be associated with clinically significant bleeding. The management of acute bleeding and the inhibitor eradication are the mainstay of the treatment. The outcome in pediatric patients seems more favorable than in adults because the inhibitors usually resolve more quickly and in a higher rate of patients. The epidemiology, diagnosis, clinical course, and management of this hemorrhagic disorder in children will be addressed in this systematic review. Pediatr Blood Cancer. 2010;55:606–611. © 2010 Wiley‐Liss, Inc.