[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 hemiver-tebra resection and short segment instrumented fusion for congenital hemivertebra malformation from December 2016 to June 2020.The clin-ical 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 post-operatively and at the latest follow-up.[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.