BACKGROUND CONTEXT Full segmented hemivertebra with large growth potential can lead to the early rapid progress of spinal deformity. Hemivertebral resection and fixation is an effective method for the treatment of congenital spinal deformity. But there were few studies focusing on the long-term follow-up of the surgical outcome and impact on spinal growth of undergoing hemivertebra resection and short-segment fusion for congenital hemivertebrae under 5 years old. PURPOSE To evaluate the effect of hemivertebra excision and short segment fixation and fusion for congenital spinal deformity under 5 years-old and its impact on spinal growth. STUDY DESIGN/SETTING A retrospective study. PATIENT SAMPLE A consecutive series of 20 patients under 5 years old with hemivertebra deformity undergo hemivertebra resection and short-segment fusion between 2004 and 2008. OUTCOME MEASURES Surgical characteristics, including fusion levels, estimated blood loss, operation duration, main curvature scoliosis, thoracic kyphosis (TK), lumbar lordosis (LL), sagittal vertical axis (SVA). METHODS All cases were 1-level full segmented hemivertebra and coronal main curve >35° and received a minimum 10-year follow-up. Clinical and imaging data were collected for statistical analysis and evaluation of surgical outcome and impact on spinal growth. RESULTS They were retrospectively studied with a mean follow-up of 11.8 years. There were 12 cases of thoracic hemivertebra and 8 cases of lumbar hemivertebra, mean aged 3.8 years (range, 2.4-5 years). The mean fused segment were 2.1. The mean operative time was 161 min (range: 120 to 320 min). The mean estimated blood loss was 175mL (range: 100 to 400 mL). The Cobb angle of coronal main curve was 39.7° (35-54°) before surgery, 5.4° (0-13°) after surgery, and 6.3° (2-16°) at final follow-up. At final follow-up, the TK angle of thoracic hemivertebra group and lumbar hemivertebra group were 22.4° (6-35°) and 25.6° (8-38°), respectively; the LL angle of lumbar hemivertebra group with 72.1° (58-80°) was significantly larger than thoracic hemivertebra group with 35.5°(24-46°). The SVA of thoracic hemivertebra group and lumbar hemivertebra group were 1.32 cm and 2.1 cm. The height of fixed segmental vertebral body, transverse and longitudinal diameter of fixed segment spinal canal is 92%, 93%, 87% of adjacent segment, respectively. CONCLUSIONS Hemivertebra excision and short-segment fusion for congenital scoliosis under 5 years old can achieve reliable fixation, satisfactory deformity correction and maintained for a long term. It has no significant impact on vertebra growth in fixed segment, but there is more likely to lead to increased lumbar lordosis in lumbar hemivertebra patient which may be related to tethered effect on spine during spinal growth. FDA DEVICE/DRUG STATUS This abstract does not discuss or include any applicable devices or drugs.