The discovery of either a dermatomal cutaneous nevus or a spinal arteriovenous malformation (AVM) should raise the suspicion of Cobb syndrome. The Cobb syndrome is a neurocutaneous syndrome in which there are metameric cutaneous and spinal AVMs. The authors present the case of a patient with acute cervical myelopathy and subtle cutaneous hemangiomas in whom a cervical perimedullary fistula was discovered. They also acknowledge Harvey Cushing's contribution to the recognition of this syndrome.