Two cases of cerebral arterial occlusive disease with telangiectasia are described. The patients are white women (ages 39 and 25), who developed symptoms following prolonged use of oral contraceptives. Their courses were slow but progressive. This condition with its characteristic angiographic picture was originally reported in Japan as a variety of cerebral arterial occlusive disease with predilection for young women. Such cases have been reported following nonspecific arteritis, meningitis, radiation therapy, trauma, and in association with neurofibromatosis. A slowly progressive occlusion in the supraclinoid portion of the internal carotid artery of young patients may be the primary lesion. In the two cases described here, prolonged use of oral contraceptives may have been related to the occlusive disease and subsequent telangiectasia.