Objective To evaluate the efficacy of simultaneous anterior and posterior hemivertebra resection in the treatment of congenital kyphoscoliosis caused by fully-segmented hemivertebra. Methods Twenty-one consecutive cases with congenital kyphoscoliosis, which were 8 males and 13 females, underwent one-stage operation of anterior hemivertebra resection and posterior instrumentation. All of the hemivertebra were fully-segmented. The average age at surgery was 11.4 (range, 1.5-16) years old. Results The average follow-up was 23 (range, 6-50) months. The average Cobb’s angle of the coronal curve was 51.4° (30°-120°) before surgery, 16.9° (0°-54°) after surgery, and 19.5° at latest follow-up. The angle of segmental kyphosis was 37.1° (0°-95°) before surgery and 16.1° (0°-48°) after surgery, and 18.2° at latest follow-up. Apical translation was improved from 4.7 cm to 1.9 cm. The average fusion segments were 4.8 (range, 2-10) segments. Complication included pressure sore in one case, pedicle cutting by pedicle screw in one case and elongation of the curve in one case. There was no obvious pseudarthrosis, decompensation, and kyphosis deformity aggravation at latest follow-up. Conclusion One-stage hemivertebra resection has a good result in the surgical treatment of congenital kyphoscoliosis caused by fully-segmented hemivertebra, and may shorten fusion levels if performed at an earlier age. Objective To evaluate the efficacy of simultaneous anterior and posterior hemivertebra resection in the treatment of congenital kyphoscoliosis caused by fully-segmented hemivertebra. Methods Twenty-one consecutive cases with congenital kyphoscoliosis, which were 8 males and 13 females, underwent one-stage operation of anterior All of the hemivertebra were fully-segmented. The average age at surgery was 11.4 (range, 1.5-16) years old. Results The average follow-up was 23 (range, 6-50) months. The average Cobb’s angle of the coronal curve was 51.4 ° (30 ° -120 °) before surgery, 16.9 ° (0 ° -54 °) after surgery, and 19.5 ° at the latest follow-up. The angle of segmental kyphosis was 37.1 ° ( 0 ° -95 °) before surgery and 16.1 ° (0 ° -48 °) after surgery, and 18.2 ° at the latest follow-up. Apical translation was improved from 4.7 cm to 1.9 cm. 2-10) segments. Complication included pressure sore in one c ase, pedicle cutting by pedicle screw in one case and elongation of the curve in one case. There was no obvious pseudarthrosis, decompensation, and kyphosis deformity aggravation at latest follow-up. Conclusion One-stage hemivertebra resection has a good result in the surgical treatment of congenital kyphoscoliosis caused by fully-segmented hemivertebra, and may shorten fusion levels if performed at an earlier age.