Congenital scoliosis is a deformity of the developing spine that results from defects in vertebral development. The developmental etiologies may be classified as either a failure of formation, a failure of segmentation, or a mixture of these two modes of maldevelopment. Early detection and close surveillance of congenital scoliosis is critical, as a rapidly progressive curve may lead to significant deformity, pulmonary restriction, and neurologic problems if not treated. Early surgical treatment is often necessary for rapidly progressive curves.