Abstract:[Objective] To investigate the clinical effect of posterior hemivertebrae resection and short segment instrumented fusion for congenital spinal malformations in children. [Methods] A retrospective study was conducted on 21 children who received posterior hemivertebra resection and short segment instrumented fusion for congenital hemivertebra malformation from December 2016 to June 2020. The clinical and imaging data were evaluated. [Results] All the patients had operation performed successfully with no serious complications such as neurovascular injury, while with operation time of (199.3±10.7) min, intraoperative blood loss of (491.4±74.1) ml and fusion segments of (2.8±0.2). Regarding imaging, compared with those preoperatively, the coronal Cobb angle [(37.7±2.2)°, (10.7±2.0)°, (10.1±2.0)°, P＜0.001], local scoliotic Cobb angle [(32.8±2.0)°, (7.2±1.8)°, (6.1±1.6)°, P＜0.001], top vertebrae migration [(22.4±2.8) mm, (11.3±1.5) mm, (7.2±0.9) mm, P＜0.001], cephalad contralateral compensatory [(15.9±1.9)°, (6.2±1.1)°, (4.7±0.8)°, P=0.002], caudal compensatory [(18.6±1.8)°, (7.8± 1.3)°, (5.7±1.1)°, P＜0.001], and local kyphotic angle [(19.0±3.0)°, (7.9±1.4)°, (7.3±1.4)°, P＜0.001] significantly reduced immediately postoperatively and at the latest followup. [Conclusion] Single posterior hemivertebra resection and short-segment instrumented fusion for congenital scoliosis deformity in children have advantages of short operation time, less intraoperative bleeding, satisfactory correction, do achieve good clinical consequences.