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In analysing and investigating 66 consecutive cases of congenital scoliosis, we aimed to evaluate the clinical pattern of presentation, identify the underlying vertebral anomaly causing the deformity, and determine the frequency of associated intraspinal anomalies, cardiovascular anomalies and genitourinary anomalies. Associated general anomalies were seen in 44% of the cases. The commonest pattern of deformity was a right-sided thoracolumbar curve. Hemivertebra was the most common vertebral anomaly (45%). Occult intraspinal anomalies were seen in 15% of cases, diastematomyelia being the commonest anomaly. Genitourinary anomalies and cardiovascular abnormalities were encountered in 6% and 15% of patients respectively. As decision making in the treatment of congenital scoliosis depends on the natural history of progression of the deformity and associated intra and extra spinal anomalies, identification of all associated anomalies is essential.