The purpose of this study was to assess the surgical outcomes of posterior vertebral column resection (PVCR) with short-segment fusion for pediatric patients with congenital kyphoscoliosis (CKS). The medical records of 12 consecutive pediatric patients with CKS due to hemivertebrae located in thoracolumbar and lumbar area that had undergone PVCR and presented for follow-up at a minimum of 2 years were retrospectively reviewed. The mean follow-up period was 56.2 months, and the mean age at the surgery was 9.2 years. We evaluated radiographic parameters using plain radiographs, and evaluated segmental correction using computed tomography imaging. The mean values of the preoperative Cobb angle (cranial curve, main curve, and caudal curve) were 16.0°, 41.3°, and 25.0°, respectively. The main curve was reduced 5.4° after surgery and was maintained at 6.3° at the time of the most recent follow-up. The overall correction rate of main curve was 86.6%. Spontaneous correction rate in the cranial curve and caudal curve were calculated as 55.9 and 80.8%, respectively. The mean segmental scoliosis in the osteotomized segments and fused segments at preoperative/postoperative/final follow-up (FFU) were 40.8°/7.8°/9.2° and 34.3°/3.9°/5.1°, respectively. The mean segmental kyphosis in the osteotomized segments and fused segments at the preoperative/postoperative/FFU were 36.0°/3.8°/4.0° and 27.5°/-1.3°/0.7°, respectively. Our data indicate that PVCR with short-segment fusion for CKS can provide good correction in the main curve and spontaneous correction in the compensatory curves after a minimum 2-year follow-up. Further investigation over the long term is mandatory for pediatric patients.