Diagnosis of an intracranial dural arteriovenous fistula (DAVF) with spinal perimedullary venous drainage is challenging because the presenting symptoms are usually related to dysfunction of the spine, not of the brain. Repeated spinal angiograms are usually performed before the diagnosis is finally made by cerebral angiography. We report two cases of intracranial DAVFs with spinal perimedullary venous drainage. In both cases contrast-enhanced cervical MRI demonstrated dilated lower brainstem and upper spinal veins, which, we believe, is a good indicator of the existence of such drainage. We suggest that, in cases with perimedullary serpentine enhancement on thoracic or lumbar MR images, additional Gd-enhanced cervical spinal MR imaging should be performed. The simple process of tracing the veins upwards may avoid a lot of unnecessary examinations and delay in the diagnosis.