Neurofibromatosis associated with vascular disorders is generally known as a vascular neurofibromatosis. In the neurosurgical aspect, it has been pointed out that "moyamoya disease" -like stenosis and/or occlusion of the internal carotid artery are seen in some of the patients with neurofibromatosis. This 58-year-old female with multiple café-au-lait spots and neurofibromas all over the body was admitted to our hospital with complaints of left orbital tumor and tinnitus. She had been suffering from the left intraorbital neurofibroma since ten years of age. Radiological examinations revealed left intraorbital neurofibroma extending into the intracranial cavity, multiple aneurysms and arteriovenous fistula. The aneurysms were located at the left vertebral-posterior interior cerebellar artery junction and at the extracranial internal carotid artery. The arterio-venous fistula was demonstrated at the left extracranial vertebral artery. Pathological changes of the blood vessels in the vascular neurofibromatosis are essentially obstructive lesions due to intimal thickening. Only few cases of neurofibromatosis associated with aneurysms and arteriovenous fistula have been reported, and eleven cases of them could be reviewed in the literature. Six are with intracranial aneurysms, two with extracranial aneurysms and three with extracranial vertebral arteriovenous fistula,. It is interesting that in all cases the extracranial vertebral arteriovenous fistula are located on the left side. This is corresponding to the fact that the bone defect around the asterion is predominantly seen on the left side in cases of neurofibromatosis.