Congenital anomalies of the spine are common in small animals. The type of deformity, location, severity, time of onset of associated clinical signs, and progression of neurologic dysfunction varies widely. To promote clearer understanding, the authors present the various spinal malformations using modified human classification schemes and use current widely accepted definitions and terminology. The diagnostic approach, including utilization of advanced imaging, and surgical management is emphasized. Copyright 2010. Published by Elsevier Inc.