To study the clinical characteristics and surgical treatment of congenital scoliosis with diastematomyelia, four patients were found to have scoliosis with diastematomyelia by CT and MRI.The first step of surgery was to excise the osteal compartion in spinal cord, the second step was the posterior scoliosis correction and fusion after two weeks. All the patients had successful surgery. The body height of the patients increased 4 75cm and the Cobb angle deceased 43? in average. No complication was found in the nervous system. This method is very effective, and is worth popularizing its application.