Cerebrovascular lesions in neurofibromatosis are uncommon. Cerebral arterial occlusive diseases associated with neurofibromatosis have been documented in less than 40 patients in world literature. We report a 28-year-old male patient with moyarnoya disease associated witb neurofibromatosis. Neurofibromatosis manifestation consisted of multiple cafe-au-lait spots, depigmentation, neurofibromas, iris Lisch nodule, axillary freckling and mental retardation. On admission with acute ischemic syndrome, focal neurologic deficits were decreased left sided pinprick sensation and bilaterally brisk knee jerks. Magnetic resonance imaging showed multifocal ischemic lesions in deep cerebral white matter and multifocal signal voids at the basal ganglia. Angiogram showed bilateral occlusion of distal internal carotid artery associated with moyamoya vessels. To our knowledge. This is a first case report of moyarnoya disease with neurofibromatosis in Korean literature.