Congenital scoliosis is the most frequent congenital deformity of the spine. Congenital curvatures are due to anomalous development of the vertebrae (failure of formation and/or segmentation). Congenital scoliosis is believed to be related to an insult to the fetus during spine embryological development, and associated malformations (heart, spinal cord, kidney.) are frequently observed. A perfect understanding of the natural history of the deformity and the treatment principles will allow best management of these complex spine deformities. New imaging techniques like three-dimensional computed tomography (CT) and magnetic resonance imaging (MRI) are important tools for analyzing the underlying deformity and understanding the evolution of the complex deformities. The mainstay of treatment is either observation or, in case of curve progression (>10 degrees /year), surgery. Different surgeries are described with two main principles: (1) prophylactic surgeries like hemiepiphysiodesis or in situ fusions that will prevent worsening or allow progressive correction over time, and (2) corrective surgeries, with spinal fusion with or without spinal resection. Exceptional procedures (e.g. spinal column resection or halo distraction) can be attempted in cases of very severe deformity. Congenital curves must be carefully observed to choose the least invasive procedure at the right time and to minimize spinal cord risks.