A 73-year-old man presented with a rare transverse sinus dural arteriovenous fistula (dAVF) with venous reflux to the brainstem and medulla manifesting as brainstem and spinal cord edema mimicking brainstem infarction. Complete occlusion of the fistula was achieved by transvenous embolization, resulting in angiographic cure of the fistula and progressive improvement of the symptoms. Intracranial dAVFs with perimedullary venous drainage, type V according to the Cognard classification, are rare lesions with distinctive clinical, radiological, and therapeutic aspects. This case demonstrates that the symptoms of dAVF with perimedullary venous reflux are variable, so dAVF should be considered in patients with clinical and radiological findings suggestive of congestion in the brainstem and spinal cord. Dysfunction of the medulla and spinal cord caused by venous hypertension is the most probable cause of the neurological symptoms in such cases. Interventional therapy can lead to angiographic cure and resolution of the symptoms.