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We report a case of a skeletally immature achondroplastic adolescent with significant thoracolumbar lordosis who presented with neurogenic claudication and urinary incontinence progressing over a 1-year period. She underwent decompressive lumbar laminectomy from T12 to L5 with preservation of the facet joints. Over the ensuing 4 years of follow-up, she developed a progressive thoracolumbar kyphosis that progressed to 105 degrees. She remained neurologically intact and was fully ambulatory, but had severe back pain in the area of the deformity. Because of the severity and progression of her deformity, a combined anterior and posterior fusion and stabilization was required. We present this case and a review of the literature on spinal stenosis in achondroplasia and the complication of post-laminectomy kyphosis.