Neurofibromatosis produces a broad spectrum of clinical manifestations. Cafe-au-lait spots, cutaneous neurofibroma and tumors of the central and peripheral nervous system are well known manifestations. One of the more serious aspects of the disease relates to the arterial involvement. Renal arterial disease with resultant hypertension has been paticularly well documented. However, cerebrovascular lesions in neurofibromatosis are uncommon. Cerebral arterial occlusive diseases with juxtabasilar telangiectasia (moyamoya disease) associated with neurofibromatosis have been documented in about 40 patients in the world literature. We report a case of neurofibromatosis associated with the moyamoya disease.