A 61-year-old man presented with a rare case of spontaneous vertebral arteriovenous fistula manifesting as radiculopathy of the left arm. MRI demonstrated an abnormal dilated vascular space on the left ventral aspect of the spinal canal and compression of the spinal cord and subarachnoid space. MRA disclosed a single high-flow vertebral arteriovenous fistula. Angiography showed a direct high-flow fistula at the C2-3 level between the left vertebral artery and the spinal extradural veinous plexus, and an abnormal dilated left vertebral artery with "string of beads"-like feature. The fistula was successfully obliterated by coil embolization with preservation of patency of the left vertebral artery, resulting in improvement of the signs and symptoms. Retrospectively this spontaneous vertebral arteriovenous fistula was considered in association with fibromuscular dysplasia.