A single lumbar hemivertebra can be expected to cause progressive scoliosis. The aim of this study was to evaluate the results of a lumbar hemivertebra resection and short-segment fusion through a combined posterior and anterior approach.From 1987 to 2002, a consecutive series of twenty-one patients with congenital scoliosis or kyphoscoliosis due to a lumbar hemivertebra were managed by resection of the hemivertebra through a combined posterior and anterior approach and with the use of a short anterior and posterior convex-side fusion.The mean age at the time of surgery was 3.3 years (range, twelve months to 10.2 years). The mean followup period was 8.6 years. There was a mean improvement of 71.4% in the segmental scoliosis curve from a mean angle of 32.9 degrees before surgery to 9.4 degrees at the time of the latest follow-up assessment, and a mean improvement of 63.9% in the global scoliosis curve from 34.1 degrees to 12.3 degrees. The mean final lordosis was within normal values.Excision of a lumbar hemivertebra is safe and provides stable correction when combined with a short-segment fusion. Surgery should be performed as early as possible to avert the development of severe local deformities and prevent secondary structural deformities that would require a more extensive fusion later.