Spondylometaphyseal dysplasia(SMD) is very rare disease characterized with platyspondyly and metaphyseal changes in the long bone. Shrot status and later abnormal gait associated with coxa vara are common clinical feature. A 6 years 4 months old boy progressive bilateral coax vara were transferred with abduction limitation of the hip. With the skeletal survey, multiple dysplastic changes of the metaphysic of the long bone including knee, shoulder, ankle, and flatterning of the body of lower thoracic and upper lumbar spine(T11,12,L1) were noted. A corrective valgus osteotomy on the both hip were followed with improvement of the range of motion.