BACKGROUND Congenital scoliosis due to hemivertebrae usually progresses during further growth and leads to severe deformities. Early correction in young children is therefore required. METHODS Thirty-six hemivertebrae in 33 children aged 1 to 6 years (average 3 years 5 months) underwent surgical intervention. Mean follow-up was 4.5 years (2 months to 13 years). METHODS The hemivertebra was resected by a posterior approach. The gap after resection was closed by compression via a transpedicular instrumentation, thus correcting the scoliotic deformity. RESULTS Mean Cobb angle of the main curve was 45.9 degrees preoperatively, 11.9 degrees postoperatively, and 9.9 degrees at latest follow-up. The compensatory cranial curve improved spontaneously from 18.4 degrees preoperatively to 5.0 degrees postoperatively and 3.7 degrees at latest follow-up. The compensatory caudal curve improved from 21.3 degrees to 6.7 and 5.4 degrees. The angle of kyphosis was 22.8 degrees preoperatively, 8.9 degrees postoperatively, and 6.8 degrees at latest follow-up. There was one infection, 2 pedicle fractures, and 3 implant failures. In 3 patients additional operations were performed due to new developing deformities. CONCLUSIONS Correction surgery of congenital scoliosis should be performed early before the development of severe local deformities and secondary structural changes. Posterior resection of the hemivertebrae with transpedicular instrumentation allows for early intervention in very young children. Excellent correction in both the frontal and sagittal planes, and a short segment of fusion allow for normal growth in the unaffected parts of the spine.