Devolopmental coxa vara is a rare devolopmental abnormality with an unknown etiology; characterized by an abnormal decrease in the femur neck-shaft angle, shortening of the femoral neck, relative overgrowth of the greater trochanter and shortening of the affected lower limb. Unilateral cases present with painless limp and bilateral cases present with a waddling gait and increased lumbar lordosis. Plain antero-posterior pelvis radiographs demonstrate decreased neck-shaft angle and vertikal orientation of the proximal femoral physis. Triangular piece of bone in the medial femoral neck is a characteristic radiological finding of devolopmental coxa vara but not seen in all cases. The purpose of treatment is to provide healing of the defective femoral neck, restore the limb length and neck-shaft angle to normal values, and also restore normal hip biomechanics for hip abductors to function normally. Conservative methods like abduction splinting and traction has no role in the treatment of this disorder. The only effective treatment is a valgus producing osteotomy done either at the intertrochanteric or subtrochanteric level. Indications for surgical treatment are the degree of H-E angle and presence of symptoms. In order to prevent recurrence after surgical treatment, H-E angle must be reduced below 38–40º.