We have recently encountered a case of high cervical arteriovenous malformation which caused subarachnoid hemorrhage. A 54-year-old male was admitted to our hospital due to sudden onset of severe headache. Neurological examination revealed right sixth nerve palsy which suggested elevated intracranial pressure. A CT scan showed subarachnoid hemorrhage predominantly in the posterior fossa, massively ventral to the brain stem, with reflux into the fourth ventricle. No abnormal vascular lesions were found in the first cerebral angiography which included four vessels and bilateral thyrocervical trunks. Ten days later, the second cerebral angiography demonstrated an arteriovenous shunt via the right vertebral artery draining into the epidural space of the upper cervical region. A suboccipital craniotomy and laminectomy of the atlas were performed. An abnormally dilated vein along the right C1 nerve ventral root which drained into the tortuous dilated coronary venous plexus on the ventral side of the spinal cord was found. We made a diagnosis of dural arteriovenous malformation fed by the C1/C2 radicular artery via the right vertebral artery and draining into the radiculomedullary vein and the epidural venous plexus. The interruption of both intra- and extradural draining veins was carried out. The postoperative course was uneventful.