We described a dissecting aneurysm of the vertebral artery (VA), which was associated with neurofibromatosis type 1 (NF1). A 41-year-old man was referred to our hospital because of abrupt, severe headache. A CT scan revealed diffuse subarachnoid hemorrhage (SAH) predominantly in the prepontine cistern. The angiograms showed a string sign in the left VA, just distal to the posterior inferior cerebellar artery (PICA). The vertebral dissection was considered responsible for SAH, and endovascular occlusion of the left VA was attempted. During the intervention, the patient complained of severe neck pain at the time of selective vertebral angiography, which revealed an arteriovenous fistula. The VA was occluded proximal to the PICA with GDC, which covered the fistula. Open surgery confirmed the two unruptured aneurysms. Intracranial dissection is rarely reported in association with NF1. However, ateriovenous fistula is not an uncommon combination with dissecting aneurysm and the extracranial segment of the VA is a characteristic target. Anatomical feasibility is conceivably the pathogenesis.