Background: Reports on associated intraspinal and other organ anomalies in congenital scoliosis are variable. We are reporting such findings in two orthopaedic referral centers in Tehran-Iran.Methods: During a period of 15 years, in a case series study, 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. Tethered cord syndrome was the most common neurologic problem in 38 patients (45.8%). Anomalies in other parts of body detectable by physical examination were observed in 155 patients, and detected by help of clinical evaluation in 121 and 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 extremities, 14 Urogenital, 10 heart and 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.