Congenital scoliosis is a deformity characterized by longitudinal and rotational imbalance caused by formation disorder and malsegmentation of embryonic vertebral bodies. Hemivertebral deformity caused by the former is the main cause of congenital scoliosis. The etiology of CS is relatively complex and unclear. In recent years, there have been many researches on the etiology of CS, but it is agreed that heredity and environment are the main pathogenesis factors. Advance in genetic and environmental factors in the pathogenesis of CS will be reviewed in this article.