Vascular changes in neurofibromatosis are most commonly described in the renal arteries. In the present study, two children with neurofibromatosis and cerebral vascular occlusive changes demonstrated by cerebral angiography are reported. Although focal neurological findings in children with neurofibromatosis are often due to tumours, the sudden development of neurological symptoms in such cases should alert paediatricians to the possibility of cerebral vascular disease.