We report five cases of intracranial dural arteriovenous fistula (DAVF) with perimedullary venous drainage. All the patients presented with rapidly progressive myelopathy and three had autonomic disorders. The DAVF were on the tentorium cerebelli (two cases), sigmoid (one), superior petrosal (one), and cavernous sinus (one). Slow venous drainage was directed through dilated perimedullary cervical veins. The transverse sinus was occluded in two cases. MRI, performed in four cases, demonstrated high signal on T2-weighted spin-echo sequences in the medulla oblongata and upper cervical spinal cord consistent with oedema, which signal resolved after complete cure of the DAVF in three cases. Embolisation was performed in all cases. It was followed by clinical deterioration in two cases and in the dramatic improvement in the other three, with complete clinical cure in two. Extensive venous thrombosis may explain the deterioration observed in one case.