A 33-year-old female with neurofibromatosis and intracranial vascular lesions of the Moya-Moya type is reported. Clinical and angiographic aspects of both syndromes are discussed with respect to earlier reports of this rare association, with the conclusion that it produces slow intelectual and motor deterioration in adolescents or young adults. There are no specific guidelines to therapy, and the prognosis is mixed, some patients ceasing to progress at least for a few years.