A 53-year-old man abruptly developed headache and unconsciousness. Brain computed tomography (CT) and CT angiography showed subarachnoid hemorrhage, intraventricular hemorrhage, and multiple tortuous vascular structures on the brain stem and upper cervical spinal cord. Four-vessel angiography displayed intradural ventral arteriovenous fistula, supplied by the left vertebral and occipital arteries. Drainage was via both sigmoid sinus and cervical venous plexus. He had been treated with transarterial coil embolization of the left vertebral artery. Subsequently, he suffered from left hemiplegia and cognitive problem. Brain magnetic resonance (MR) and MR angiography performed 4 weeks later revealed multiple infarctions on the left cerebellum, left upper cervical spinal cord, and both medial thalamus, as well as occlusion of the left vertebral artery with reduction in varix size. After rehabilitative management, his muscle strength and cognitive function improved. We report a very rare case of dural arteriovenous fistula on the brain stem and upper cervical spinal cord.