Congenital scoliosis is the most frequent congenital deformity of the spine.The congenital curvature is caused by early embryologic errors in vertebral column formation and/or segmentation. This is usually concurrent with errors in development of additional organs; the surgeon should look for associated renal, cardiac, auditory, genitourinary anomalies and Intraspinal abnormalities. New imaging techniques like three-dimensional computedtomography and magnetic resonance imaging are important tools for analyzing the underlying deformity and understanding the evolution of the complex deformities. Curve progression is best documented by measuring identical landmarks on sequential radiographs. 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. Early detection andclose surveillance of congenital scoliosis is critical, as a rapidly progressive curve may lead to significant deformity, pulmonary restriction, and neurologic problems if not treated. The mainstay of treatment is surgery in case of curve progression (>10°/ year). Different surgeries are described:(1) prophylactic surgeries like hemiepiphysiodesis or in situ fusions that will prevent worsening or allow progressive correction over time, (2) corrective surgeries, like spinal fusion with or without spinal instrumentation and, spinal resection in severe cases, (3) Miscellaneous procedures: halo distraction, growing rods and expansion Thoracoplasty.Congenital curves must be carefully observed to choose the least invasive procedure at the right time and to minimize spinal cord risks, and must be followed up to bone maturity.