Ninety-four patients with moyamoya disease (56 in the pediatric age group and 38 adults) were treated by revascularization surgery in the past 21 years (1979--2000). Combined surgery of the superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis and indirect revascularization of encaphalo-duro-arterio-myo-synangiosis (EDAMS) was performed. Ischemic attacks disappeared in most patients within 1 year (mean) after surgery in pediatric cases. No morbidity or mortality was experienced in the pediatric group. Some children with cerebral infarction before the surgery, however, had mild mental retardation even after the surgery. Early diagnosis and proper prophylactic surgical treatment is indispensable for pediatric patients with moyamoya. MR angiography is an important diagnostic modality for the screening and longitudinal follow-up of moyamoya disease.