Intracranial hemorrhage (ICH) in the newborn or young infant is an uncommon presenting manifestation of hemophilia. Its occurrence is almost always preceded by mild-to-moderate head trauma, unlike adult hemophiliacs in whom ICH occurs without prior head injury in 50% of cases. The bleeding event may follow a minor complication of labor or delivery (eg, prolonged second-stage labor or the use of forceps). Recent experience at our institution, a major tertiary care children's hospital, indicates that the diagnosis of hemophilia is often overlooked in a young infant presenting with ICH, a history of perinatal or postnatal head injury, and a prolonged activated partial thromboplastin time (PTT). Three young infants with hemophilia (two moderate cases and one severe case) presented with head trauma and were later found to have factor VIII deficiency. Despite prolongation of the preoperative PTT in each case, hemophilia was not considered before neurosurgery was performed. In one case, PTT prolongation was ascribed to tissue thromboplastin-induced intravascular coagulation. There was one death secondary to overwhelming intraventricular hemorrhage and iatrogenic bacterial ventriculitis. Some specific diagnostic and therapeutic recommendations are provided to assist the clinician in evaluating a child with ICH and a prolonged PTT who is in need of immediate neurosurgery.