A 27-year-old male presented with progressive ascending myelopathy leading to tetraparesis. Magnetic resonance imaging of the cervical spine showed dilated perimedullary veins and spinal cord edema. Catheter angiography demonstrated a direct carotid-cavernous fistula (CCF) with prominent pontomesencephalic and perimedullary venous drainage. Successful coil embolization of the fistula was performed with improvement of the patient's symptoms. To our knowledge, no case of a direct CCF with perimedullary drainage has been previously reported.