Intracranial dural arteriovenous fistula (DAVF) is rare and potentially life-threatening disease often presenting as vascular myelopathy. The early and proper diagnosis is challenging because the clinical manifestations are related to the distribution of the draining vein, not the fistula site, and imaging findings are similar to demyelinating disease of the spinal cord. The authors present the case of a 45-year-old man who developed acute progressive quadriplegia and respiratory difficulty with an enhancing, longitudinally extensive cervical cord lesion. These symptoms were highly suspicious for transverse myelitis but were caused by an intracranial DAVF. Intracranial DAVF with venous reflux to the brainstem and spinal cord is a rare but important differential diagnosis of progressive worsening myelopathy that is treatment resistant and gives the diagnostic impression of transverse myelitis.