The concept of a cerebrofacial arteriovenous metameric syndrome (CAMS) recently has been posited. A 22-year-old woman presented with gait ataxia. She had unilateral angiomatous lesions involving the lower face, the auricle, and the retromastoid area, as well as the tongue on the right side. Angiography revealed the presence of extensive arteriovenous malformations (AVMs) in the ipsilateral cerebellum and pons, in addition to those located in the facial region. The authors report a case of multiple AVMs of the face, cerebellum, and pons, corresponding to CAMS Type 3, and include a review of the literature.