Objective To analyze the safety and efficacy of one stage spinal osteotomy for patients who had progressive severe and rigid congenital scoliosis (CS) associated with split spinal cord malformation (SSCM).Methods A total of 24 patients underwent one stage spinal osteotomies for severe and rigid CS associated with SSCM from September 2007 to November 2010 in our hospital.11 of these patients were males and 13 were females with an average age of 15.6±3.3 years (range,12-28 years).There were 11 patients with Type Ⅰ SSCM and 13 patients with Type Ⅱ SSCM.The mean major coronal curve ranged from 80° to 135° (average,93.4°±13.9°) and the coronal flexibility ranged from 4.9％ to 28.3％ (average,13.9％±7.1％).Before the corrective stage of surgery,bony spurs were resected in patients of Type Ⅰ SSCM,while nothing was done to the Type Ⅱ SSCM.Then,posterior osteotomy and fusion was performed to correct the spinal deformity.Results All patients were followed up for a minimum of 24 months after initial surgical treatment with an average follow-up of 44.5±17.4 months (range,24-68 months).The average operation time was 554.7±118.4 min (range,395-895 min) and the average blood loss was 3 741.7±2 260.0 ml (range,1 000-9 600 ml).The average amount of blood transfusion was 3 711.3±2 059.4 ml (range,800-8 850 ml).The immediate postoperative correction rate was 47.2％ to 96.7％ (average,65.9％± 11.3％).At the final follow-up,the final correction rate was 62.7％± 12.0％ (range,40.7％-94.5％),with a correction loss of 3.0％±2.4％ (range,0.3％-8.9％).The overall major surgical complications rate was 25.0％ (6/24),including neurological deterioration in 3 patients (12.5％,3/24),cerebrospinal fluid leakage in 2 patients (8.3％,2/24) and pleural rupture in 1 patients (4.2％,1/24).The neurological deterioration recovered to the preoperative neurological status at 1 week,3 months and 6 months postoperatively and improved at the final follow-up,compared with the preoperative status.Conclusion One stage spinal osteotomy is safe and effective for severe and rigid CS with SSCM without increasing the complication rate.However,it results in longer operative time and more blood loss.