The diagnosis of congenital scoliosis encompasses a spectrum of spinal deformities that arise from a failure of formation or segmentation (or a combination of both) of the spine. The natural history, risk of progression, and treatment options are varied and unique to the type of congenital deformity, growth remaining, and location of the congenital abnormality in the spine. Careful assessment of the congenital abnormality present, knowledge of the expected course of the deformity, close observation of the spine over time, and a reasonable outcome expectation are all important to determine the optimal treatment for patients with this condition.