We report a rare case of progressive myelopathy caused by intracranial dural arteriovenous fistula with venous drainage into the spinal perimedullary veins. A 45-yr-old man developed urinary and fecal incontinence and muscle weakness in the lower limbs. Magnetic resonance imaging revealed brainstem, edema and dilated veins of the brainstem and spinal cord. Cerebral angiography showed a dural arteriovenous fistula fed by the neuromeningeal branch of the left ascending pharyngeal artery. Occlusion of the fistula could be achieved by embolization after a diagnostic and subsequent therapeutic delay. There was no improvement in clinical condition. For the neurologic outcome of these patients it is important that fistula must be treated before ischemic and gliotic changes become irreversible.