Cobb syndrome consists of a vascular skin nevus associated with an angioma in the spinal cord. We describe a young man with this condition. To our knowledge, this is the 28th case reported. Cobb syndrome must be differentiated from other syndromes characterized by vascular cutaneous lesions associated with central nervous system abnormalities, such as Sturge-Weber, Osler-Weber-Rendu, Fabry-Anderson, von Hippel-Lindau, and ataxia telangiectasia.