We report on a case of Cobb syndrome, a rare metameric disorder, characterized by a vascular abnormality of the spinal cord, with an associated vascular skin lesion of the same metamere, in an 8-year-old girl presenting with leg weakness. Magnetic resonance imaging revealed a spinal arteriovenous malformation at the level of T(10)-L(5). This finding, together with a large, pigmented nevus at this level, is diagnostic for Cobb syndrome. A systematic review of the literature on Cobb syndrome, with a focus on treatment and outcomes, is presented. Current treatment options include combinations of embolization, neurosurgical intervention, corticosteroid therapy, and radiotherapy.