Moyamoya disease is a rare entity consisting of bilateral stenosis or occlusion of internal carotid arteries with abnormal collateral vessels at the base of the brain. We present five Turkish children with this disease, which is more common in Japan. Focal neurologic deficits and/or epilepsy were the common presenting symptoms in two girls and three boys between the ages of 1.5 and 11 years. Multiple cerebral infarcts were diagnosed in all of the cases by Computed Tomography (CT). Abnormal net-like vessels at the base of the brain were detected in three patients. Cerebral angiography, which is necessary to confirm the diagnosis, showed moyamoya vessels and bilateral stenosis or occlusion of the internal carotid arteries in all cases. Although the angiographic staging was advanced in three patients, neither clinical status nor parenchymal abnormalities detected with CT were different from the other two cases.