Background: Reports on associated intraspinal and other organ anomalies in congenital SCOLIOSIS are variable. We are reporting such findings in one of the orthopaedic referral centers in Tehran.Methods: A series of 381 patients with congenital SCOLIOSIS were evaluated for intraspinal abnormalities with physical examination, radiographs, MRI and/or myelograms. They were also assessed for cardiac urogenital or anomalies in other areas by physical exam, sonography and echocardiography.Results: Cord anomaly was present in 83 (21%) cases, 26 of whom had neurologic abnormality. The third cord syndrome was the most common neurologic problem in 38 patients (45.8%), followed by diastematomyelia in 37 cases. Anomalies in other parts of body detectable by physical examination were observed in 155 patients, and detected by help of paraclinical tests in 34 patients. 84 cases had trunk anomalies, 4 genitourinary and 10 heart anomaly, 37 head and neck, 7 maxillofacial, 3 upper and 23 lower extremity, 8 cases of gastrointestinal anomalies were also detected.Conclusion: The anomalies of nervous system associated with congenital SCOLIOSIS are often without clinical manifestations. Cardiac and urogenital anomalies are not very common in congenital SCOLIOSIS.