Congenital scoliosis is an intrauterine deformity of the spine due to defects in formation and / or segmentation during embryogenesis. It may cause serious deformity, lung and cardiac problems in early childhood. Early diagnosis and treatment is important because of the difficulty in carrying these problems to the later stages of life. Congenital scoliosis is a complex spinal problem associated with many other anomalous findings. In this article, etiology, accompanying anomalies, classification, natural course and treatment of congenital scoliosis heve been discussed.