Clinical features of ten Japanese children with cerebrovascular Moyamoya disease are reported and non-Japanese paediatric cases of the disease are reviewed from the world literature. The most common initial manifestations were headache in four cases (40%), motor deficit and convulsion in three cases (30%). As their recurrent and/or residual symptoms, eight children (80%) developed motor deficit consisting of hemiplegia in five cases and paresis or weakness of the extremities in three cases, and four (40%) had headaches. The mode of presentation in our cases was similar to that of non-Japanese cases, in addition to a female preponderance. Electroencephalographic findings of prominent high voltage delta bursts following hyperventilation and slowness of returning to the normal pattern, seen in all hyperventilated cases, is one of the features. Of 15 carotid arteries visualised in ten patients, sites of occlusion or stenosis were seen between the bifurcation of the posterior communicating artery and that of the anterior cerebral artery or the middle cerebral artery in 13 arteries, with a vascular network in the basal ganglia. This study suggests that if hyperventilation procedures produce prominent high voltage delta bursts during electroencephalography in children with headache and/or motor deficit, cerebrovascular disease, especially Moyamoya disease, should be suspected.