We present a case of metameric arteriovenous malformation (AVM) in a neonate. The neonate appeared normal, except for a dimple and blue discoloration in the lower thoracic spine. Spinal ultrasonography revealed cutaneomeningiospinalangiomatosis in the thoracic spine. Spinal magnetic resonance imaging revealed a definite metameric AVM at the T10-T11 level and a cutaneous hemangioma at T11. The neonate was conclusively diagnosed with spinal metameric AVM at the T10-T11 level. The neonate was asymptomatic with no, abnormal physical or neurological complications, including urinary incontinence, fecal incontinence, or muscle weakness.