Objective To analyze the cl inical features of scol iosis associated with Chiari I malformation in adolescent patients, and to explore the val idity and safety of one-stage posterior approach and vertebral column resection for the correction of severe scol iosis. Methods Between October 2004 and August 2008, 17 adolescent patients with scol iosis associated with Chiari I malformation were treated with surgical correction through posterior approach and pedicle instrumentation. There were 9 males and 8 females with an average age of 15.1 years (range, 12-19 years). The MRI scanning showed that 16 of 17 patients had syringomyel ia in cervical or thoracic spinal cord. Apex vertebra of scol iosis were located atT7-12. One-stage posterior vertebral column resection and instrumental correction were performed on 9 patients whose Cobb angle of scol iosis or kyphosis was more than 90°, or who was associated with apparent neurological deficits (total spondylectomy group). Other 8 patients underwent posterior instrumental correction alone (simple correction group). All patients’ fixation and fusion segment ranged from upper thoracic spine to lumbar spine. Results The operative time and the blood loss were (384 ± 65) minutes and (4 160 ± 336) mL in total spondylectomy group, and were (246 ± 47) minutes and (1 450 ± 213) mL in simple correction group; showing significant differences (P lt; 0.05). In total spondylectomy group, coagulation disorder occurred in 1 case, pleural perforation in 4 cases, and lung infection in 1 case. In simple correcction group, pleural perforation occurred in 1 case. These patients were improved after symptomatic treatment. All patients were followed up 24-36 months (32.5 months on average). Bony heal ing was achieved at 6-12 months in total spondylectomy group. No breakage or pull ingout of internal fixator occurred. The angles of kyphosis and scol iosis were significantly improved at 1 week after operation (P lt; 0.01) when compared with those before operation. The correction rates of scol iosis and kyphosis (63.4% ± 4.6% and 72.1% ± 5.8%) in total spondylectomy group were better than those (69.4% ± 17.6% and 48.8% ± 19.3%) in simple correction group. Conclusion Suboccipital decompression before spine deformity correction may not always be necessary in adolescent scol iosis patients associated with Chiari I malformation. In patients with severe and rigid curve or apparente neurological deficits, posterior vertebral column resection would provide the opportunity of satisfied deformity correction and decrease the risk of neurological injury connected with surgical correction.