Spinal dural arteriovenous fistulas (DAVFs) are the spinal vascular malformations that are encountered most often, and they are usually encountered in the lower thoracic region. Cervical spine DAVFs are exceedingly rare and may be difficult to differentiate from radicular arteriovenous malformations, epidural arteriovenous shunts, or perimedullary AVFs. Typical angiographic findings in spinal DAVFs include a slow-flow shunt with converging feeding vessels from radiculomeningeal arteries draining via a radicular vein centripetally into perimedullary veins. The MR imaging findings such as spinal cord edema and perimedullary dilated vessels may be used to direct the spinal angiography that is needed to localize and classify the shunt. When the shunt is distant from the pathological imaging findings, the diagnosis may be difficult to establish, especially when the shunt is present at an atypical location such as the cervical spine. The authors present the case of a 51-year-old man presenting with lower thoracic and conus medullaris congestive edema due to a cervical spine DAVF that was located at the C-5 level. Transarterial embolization with N-butyl cyanoacrylate closed the proximal vein and completely obliterated the fistula. Clinical and imaging follow-up confirmed occlusion of the fistula, with improvement in clinical symptoms.