[Objective] To investigate the efficacy of short segment pedicle screw internal fixation for congenital scoliosis. [Methods] 21 children with congenital hemivertebrae malformation were investigated. There were 5 cases of thoracic hemivertebrae, 4 cases of thoracolumbar hemivertebrae, 11 cases of lumbar hemivertebrae, and 1 case of lumbosacral hemivertebrae. A single posterior approach was used to remove the hemivertebrae, and short segment pedicle screw fixation was performed at the same time. Full-length posteroanterior and lateral films of standing spine were taken. Imaging parameters were measured to determine the improvement of coronal and sagittal planes. [Results] The operation time was 125-360 minutes, with an average of 199.3 minutes. The amount of bleeding was 120-1600ml, with an average of 491.4ml. Operative fixation was 2~5 vertebral bodies with an average of 2.8. Artificial prosthesis was placed in 2 cases of lower lumbar hemivertebrae of adolescents. BDBO was performed in 1 case of lower lumbar hemivertebrae of adolescents. There were statistically significant differences in Cobb angle in coronal plane, Cobb angle in sagittal plane, Cobb angle of head and tail compensatory bending, and deviation of parietal vertebra between preoperative and postoperative imaging parameters (P<0.05). [Conclusion] A single posterior approach was used to remove the hemivertebra with ultrasonic osteotome and fix the congenital scoliosis deformity in children. It took short operation time, less intraoperative bleeding. The orthopedics were good, and the follow-up effect was satisfactory.