Abstract Seventeen patients with basal occlusive disease have been seen over the past 4 years. Nine of these had a classical moyamoya appearance on angiography. Of these nine, seven were adults and two were children. Eight had ischemic episodes, and one had a hemorrhage. One had neurofibromatosis with a chiasmal glioma and had received radiotherapy. Eight patients underwent superficial temporal-middle cerebral artery (STA-MCA) bypass, and one refused operation. Six patients improved, one had a stroke on the opposite side, and one died. Eight patients had unilateral basal arterial occlusive disease. Of these, three had ischemic episodes, four had hemorrhages, and one had only headaches. Four were adults, and four were children. Five underwent STA-MCA bypass with improvement, one with headaches had an aneurysm treated, and two were not operated upon. Considerations regarding the cause, therapeutic options, and disease course in these patients are discussed.