The unusual association of a giant extracranial vertebral artery pseudoaneurysm, intracranial aneurysms, and extracranial carotid occlusion in a woman with neurofibromatosis is presented. Pain as a result of expansion of the mass in the soft tissue of the neck led to her seeking evaluation. Herniation of the mass intraspinally between the occiput and C-1 resulted in myelopathy. Following balloon occlusion of the vertebral artery, the mass and associated symptoms resolved without the need for direct resection. The salient features of these unusually associated problems are discussed.