The outcome of 8 episodes of intracranial hemorrhage in 7 patients (4 males, 3 females) with congenital factor VII deficiency was evaluated. Their levels of factor VII clotting activity (FVII:C) were less than 1 per cent (n = 3) and ranged from 1.7 to 2.3 per cent (n = 4). The onset varied from the first week (n = 2), first month (n = 3), and at the ages of 6, 11 and 12 months (n = 3). The replacement therapy of 10 ml/kg of fresh frozen plasma (FFP) every 6-12 hours for 5-7 days was given to 6 patients. Only one craniotomy for the removal of hematoma was performed. The seventh patient experienced two episodes of bleeding. First, she received 20 μg/kg of recombinant factor VIIa (rFVIIa) every 6 hours for 4 days (1,200 μg) followed by FFP in one episode. Second, a craniotomy for the removal of a 7 cm diameter hematoma was performed by giving 20 μg/kg of rFVIIa every 6 hours for 12 days (9,600 μg) followed by FFP in another episode. As a result of these treatments, 2 died and 5 survived. with sequelae, except for one who received rFVIIa. The sequelae included seizure disorder (n = 1) and hydrocephalus (n = 3). Subsequently, the surviving patients received 15 ml/kg of lyophilized fresh plasma every 3-5 days as prophylactic treatment. In conclusion, rFVIIa in the dose of 20 μg/kg every 6 hours has been shown to be effective in controlling intracranial hemorrhage in patients with congenital factor VII deficiency.