Twenty-three patients with epileptic type moyamoya disease are reviewed among 200 moyamoya disease patients. Ten boys and 13 girls aged 5 months to 12 years were followed over 6 months to 17.3 years. Six had generalized seizure and 17 had focal seizure. Operations were performed within 1 year in eight patients, within 1-3 years in five, and more than 3 years after onset in 10. Nineteen patients improved and suffered no seizure without receiving antiepileptic drugs, but four patients developed true epilepsy and three of these suffered cerebral infarction. Multivariate analyses showed that toddlers aged less than 1 year and mild or severe abnormal computed tomographic (CT) findings correlated with a bad outcome. This study showed that epileptic type moyamoya disease has the same clinical features as transient ischemic attack or infarction type. Age under 1 year and CT abnormalities indicate a poor prognosis and necessity for early reconstructive surgery.