The authors reviewed four patients with dural arteriovenous malformations in the upper spinal axis. Two were at the foramen magnum and two were lower cervical. The patients presented with subarachnoid hemorrhage, a slowly progressive cervical myelopathy, a rapidly progressive thoracic myelopathy, and tinnitus with a sixth nerve palsy. This report emphasizes the importance of studying both the intracranial dural vessels as well as the supply to the cervical spine in searching for a spinal arteriovenous malformation. Subarachnoid hemorrhage with negative cerebral angiography requires spinal angiography if there are any signs or symptoms suggesting cord or nerve root dysfunction. Embolization by an endovascular approach resulted in an angiographic cure in two patients. A combination of embolization and surgery resulted in obliteration of the arteriovenous malformation in one patient. Embolization achieved a clinical cure in one patient, and clinical improvement in two patients.