Congenital malformations of the spine can produce scoliosis, kyphosis, lordoscoliosis or kyphoscoliosis. Deformities may progress slowly or fast or may even be stable without progression. Knowledge of the natural history of such deformities is of utmost importance. Besides deformities of the spine and the thorax, a number of associated anomalies have to be taken into account, like thoracic, intraspinal, cardiac and urogenital abnormalities. Special examinations, including MRI of the entire spinal canal need to be ordered prior to beginning of treatment.Conservative treatment, including physical therapy and bracing, will not control progression of the curve. Serial casting may be offered for some types of congenital malformations in early childhood. An isolated hemivertebra is best resected by a posterior approach only at age 5 to 6. Growth guiding anterior or posterior procedures like growing rods are currently indicated for very few cases. However, the VEPTR procedure is well indicated for complex congenital deformities with or without thoracic malformations, preserving the growth potential of the spine and the thorax.Kyphotic deformities can not be treated with growth preserving surgery, so that early correction with short fusion is usually indicated. The most severe deformities may have to be treated with complex osteotomies, including VCR (vertebral column resection).