The authors report an instance of familial moyamoya disease involving a 47-year-old female and her 18-year-old daughter, both of whom initially presented, within a 1-year period, with intracranial hemorrhage. Cerebral angiography showed the typical features of moyamoya disease. The mother, whose disease was more advanced, underwent superficial temporal-middle cerebral artery bypass, with satisfactory results. Human leukocyte antigen typing was performed in both cases. The pertinent literature is reviewed.