The authors report their experience in successfully treating a 15-week-old child who became comatose following a spontaneous intracerebral hemorrhage. It was initially believed that a tumor in the right frontal lobe caused the hemorrhage. Coagulation studies revealed abnormal results on presentation, and the problem was only partially corrected after an infusion of fresh frozen plasma. The child underwent an emergency craniotomy in which the hematoma was evacuated, and a biopsy specimen was obtained from a firm mass at the base of the hematoma cavity. Postoperatively, the child recovered completely, and an analysis of detailed coagulation studies revealed that the child had a factor X deficiency. Histological analysis of the biopsy specimen revealed normal brain tissue with hemorrhagic infiltration. Subsequently, the child achieved normal developmental milestones. A diagnosis of congenital bleeding disorder should be considered in children with spontaneous intracerebral hemorrhage, even in those with no prior episode of extracerebral spontaneous hemorrhage.