The authors reported an extremely rare case of bilateral spontaneous vertebro-vertebral arteriovenous fistulas (VVAVFs) associated with fibromuscular dysplasia (FMD). A 53-year-old hypertensive woman has been placed on antiplatelet drug following diagnosis of supratentorial ischemic stroke for 2 years. Few months before hospitalization, she developed bilateral audible bruits and progressive spastic quadriparesis. Magnetic resonance imaging (MRI) of the cervical spine showed bilateral dilated epidural venous pouches, causing cervical spinal cord compression. Angiography revealed bilateral high-flow VVAVFs with diffuse ectasia, and typical "string of beads" appearance. The left-side fistula located at C2-3 intervertebral foramen and the right-side at C3-4 intervertebral foramen. Reconstitution of bilateral vertebral arteries from bilateral occipital and ascending cervical arteries was also noted distal to the fistulous site. Other multivessel involvement included bilateral extracranial internal carotid, right renal, and intracranial arteries. The right internal carotid studies showed short segment of fenestration or duplication of the distal cervical internal carotid artery (ICA) combined with a small pseudoaneurysm at the distal end of the fenestrated segment, probably representing previous arterial dissection. Under general anesthesia, the fistulas were successfully treated by bilateral V2 segments sacrifice involving fistulous sites using fibered coils. Follow-up MRI and angiography confirmed complete obliteration of the fistulas and resolution of large epidural venous pouches. The patient has gradually improved and returned to her normal daily activities within six months after treatment.