Intracranial hemorrhage (ICH) is a life-threatening complication of hemophilia. Seven of the 288 hemophiliacs living in Israel suffered eight episodes of ICH during the years 1972 to 1982. All episodes occurred in hemophilia A patients, with a higher incidence among patients with factor VIII inhibitor. Diagnosis was confirmed by computed tomographic scan in seven of the eight episodes. Four of the 7 patients died despite adequate factor replacement and supportive therapy, probably due to a conservative and hesitant neurosurgical approach. The correction of factor VIII to hemostatic level alone is inadequate in the majority of cases, and there is sudden deterioration in the patient's condition and death. Operation is strongly recommended when no improvement is noted within a few hours.