Acquired hemophilia A is a very rare, serious bleeding disorder. We describe a 5-year-old female who developed an acquired factor VIII inhibitor, and while under treatment with steroids, had an intestinal perforation with peritonitis and septic shock, making her a poor candidate for further immunosuppression. She was treated with rituximab with rapid, complete eradication of the inhibitor. She represents the first published case of a pediatric patient with acquired hemophilia A successfully treated with rituximab.