Congenital scoliosis is caused by early embryologic errors in vertebral column formation. Defining the deformity, predicting the natural history, and applying the correct treatment can help ensure successful management. Most congenital spine anomalies can be classified, and many have a predictable natural history. Because the deformities are associated with other organ system anomalies in more than half of patients, the surgeon should look for cardiac, auditory, genitourinary, and renal anomalies. Intraspinal abnormalities are present in approximately one third of patients with congenital spine deformities. Curve progression is best documented by measuring identical landmarks on sequential radiographs. Magnetic resonance imaging is warranted when curve progression is established or when surgical intervention is planned. Management of progressive deformity is generally by early in situ fusion because orthotic treatment is rarely appropriate. Other surgical techniques include combined anterior and posterior epiphysiodesis, hemivertebra resection, and reconstructive osteotomies.