The clinical and angiographic features of 46 vertebral arteriovenous fistulas (AVFs) seen during a 12-year period (45 patients) were reviewed. Fourteen patients were asymptomatic, with vertebral AVF discovered at routine clinical examination. Specific symptoms at presentation in the other patients were tinnitus (n = 21), vertigo (n = 6), neurologic deficit (n = 3), and pain (n = 2). Of the 46 AVFs, 19 (41%) were caused by trauma and 27 (59%) were spontaneous. The fistula was found at C-1 to C-2 in 21 (46%) cases, at C-2 to C-5 in five (11%), and below C-5 in 20 (44%). Thirty-four patients (35 vertebral AVFs) were treated with the endovascular technique. Embolization was performed with latex balloons filled with contrast medium in most cases. Endovascular therapy resulted in complete occlusion in 32 cases (91%) and partial occlusion in three (9%). The vertebral artery could not be preserved in three patients. Endovascular balloon treatment of vertebral AVFs is effective in occluding the shunt, avoids general anesthesia and surgical intervention, and results in minimal morbidity. Endovascular therapy is the treatment of choice for vertebral AVF.