Tentorial dural arteriovenous fistulas (TDAVFs) draining into the spinal venous system are rare lesions. The clinical presentation can be devastating and the diagnosis delayed because of the initial nonspecific imaging and laboratory findings. We report a case of a 20-year-old woman with a rapidly progressive myelopathy, who was found to have a left TDAVF, fed by a single arterial feeder with drainage into the perimedullary venous system. The fistula was surgically clipped. The patient showed neurologic improvement at her 3-month follow-up but still had significant weakness of all 4 extremities. Early diagnosis is key as the neurologic symptoms are reversible with appropriate treatment. We review the relevant literature, imaging characteristics, and treatment modalities for TDAVF.