Introduction: Cleidocranial dysplasia (CCD) is a rare inherited disorder. The main clinical features of CCD include persistent, open cranial sutures with bulging calvaria; hypoplasia or aplasia of the clavicles; dental anomalies; a short middle phalanx in the fifth finger; and associated vertebral anomalies.Congenital coxa vara is a relatively rare developmental abnormality characterized by a decreased femoral neck-shaft angle, shortening of the femoral neck, and shortening of the affected lower limb. Case presentation: A 17-year-old boy is a known case of cleidocranial dysplasia was complaining of limbing since childhood, progressive, and associated with left hip pain if he sits crossed leg for long time. He underwent a left hip (intertrochanteric) valgus osteotomy. Conclusion: orthopedic surgeons should following-up CCDpatients regularly for assessing complication and the development of abnormal gait or changes in hip range of motion, signs of “chef’s hat” a feature of femoral head deformity, coxa vara, or osteoporosis. Patients who develop coxa varamanneged by surgery, whereas patients with clavicular hypoplasia and absence of pubic symphysis ossification can usually be observed.