We report a 62-year-old woman who presented with a myelopathy at the lower thoracic level. Left vertebral angiography revealed a dural arteriovenous fistula (DAVF) at the craniocervical junction, draining into an anterior spinal vein. Below the T7 level, the spinal cord gave high signal on T2-weighted images and enhanced with Gd-DTPA. The patient was successfully treated by simple clipping of vein draining the DAVF. The abnormal signal intensity and contrast enhancement rapidly regressed, except in the conus medullaris. Regression of the parenchymal abnormality on serial MRI following treatment corresponded closely with postoperative improvement of neurological function.