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The main treatment for congenital hemivertebra is posterior hemivertebrectomy with bilateral transpedicular fixation. To date, studies describing posterior unilateral fusion are few, especially in younger children. In present book, the modified method by posterior hemivertebrectomy combined with unilateral transpedicular instrumentation and fusion was described, which is a commendable procedure. It is a simple, secure, reliable, less invasive and well tolerated technique that can successfully resolve this kind of congenital scoliosis in children.