Cobb syndrome represents the concurrent findings of a metameric spinal vascular malformation and a cutaneous vascular malformation within several dermatomes of each other. This rare entity engenders many difficult decisions with respect to appropriate therapeutic management. Historically, surgical excision carried a high morbidity, and conservative management without intervention was preferred. More recently, several cases of endovascular embolization have been reported with good success. The authors describe the case of a 17-year-old boy who presented with a right gluteal angioma and was found to have a spinal arteriovenous malformation. Multiple embolizations failed to prevent neurological deterioration, and the patient eventually became wheelchair dependent. Surgical excision of the malformation led to partial recovery of neurological function, and at the latest follow-up, 52 months postoperatively, the patient was able to ambulate independently. This case demonstrates the successful treatment of a patient with Cobb syndrome with surgical excision after multiple refractory embolizations. A multidisciplinary approach, which balances the patient's current neurological function against the risks and potential gains from any interventional and surgical procedure, is recommended.