A fenestration of the vertebral artery is considered to be an unusual vascular variation that is defined as a bypass artery along the extracranial or at intracranial segment of the artery. A fenestration of the vertebral artery associated with an arteriovenous malformation at the cerebellopontine angle is extremely rare. We report a case of the ruptured arteriovenous malformation with a fenestration of vertebral artery. The embryologic genesis and clinical significance of a fenestration of the vertebral artery are discussed.