Congenital spinal deformities develop due to the presence of congenital anomalies that impact one or more vertebrae, leading to asymmetrical vertical growth of the spine, with the curvature worsening as skeletal development progresses. In Part 1 of this review, the aetiology, classification and associated anomalies related to the congenital spinal deformity are discussed. In Part 2 of this review, the evaluation of patients presenting with a congenital spinal deformity, necessary investigations, as well as both conservative and surgical treatment strategies are discussed. Accurate and timely identification of the specific type of congenital spinal deformity and the likelihood of progression of the curve will determine the initiation of appropriate treatment. Bracing has proven ineffective in managing congenital scoliosis other than when trying to control structural compensatory curves that develop at the levels above or below the congenital vertebral defect. Surgical interventions are designed to promote balanced spinal growth and prevent both severe deformities and neurological complications. Surgical management may include in situ fusion, growth arrest techniques, hemivertebra excision, posterior correction and instrumented spinal fusion, and growth-sparing techniques, each carrying varying risks of significant medical and neurological complications. In cases of severe deformities with risk of spinal cord compression, spinal osteotomies and vertebral column resection may be necessary.