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Objective:To report the clinical outcome of surgical treatment of spinal stenosis secondary to achondroplasia, the authors review the clinical and radiological presentation. Methods:Five cases, two males and three females, of spinal stenosis secondary to achondroplasia were reviewed with medical records and radiologic study. The mean follow-up period was 26.8(range:10-72)months. Results:In four patients, stenosis was more pronounced at lumbar area. The other patient revealed thoracolumbar and lumbar stenosis with kyphosis at thoracolumbar junction. All patients suffered from neurogenic intermittent claudication. Two patients presented with paraparesis and urinary dysfunction. On radiologic evaluation, all patients showed typical short pedicle, decreased interpedicular distance and severe stenosis. Only decompressive laminectomy was performed without fusion at lumbar area in four patients. One stage posterior interbody fusion and pedicle screw fixation was performed in one case which showed thoracolumbar stenosis and kyphosis. Radicular pain and neurogenic intermittent claudication improved after surgery. Conclusion:One stage posterior lumbar interbody fusion and pedicle screw fixation might be an effective method for the thoracolumbar kyphosis in patients of achondroplasia and for the cases of potential instability following decompression. Key words:Achondroplasia;Spinal stenosis;Surgical treatment.