We report a case of intracranial dural arteriovenous (DAVF) draining into the spinal medullary veins. A 49-year-old woman presented a rapidly progressive ascending myelopathy resulting in a C3-C4 tetraplegia associated with acute respiratory failure at the twelfth hour. MRI revealed swelling of the cervical spinal cord, hyperintensity on T2 and enhancement of enlarged veins on MR angiography. A conventional angiography showed the DAVF with venous drainage into the spinal vein extending to the conus medullaris. After embolization, neurological recovery occurred during the first week, allowing tracheal extubation on day 2. Clinical, radiological and therapeutic aspects of this uncommon pathology are presented.