Popliteal pterygium syndrome is rare, with 60 cases described in the literature. The syndrome includes a popliteal web extending from the ischium to the heel and other abnormalities including toenail dysplasia, deformities of the foot and toes, and oral cavity abnormalities. The main orthopedic problem is a flextion contracture of the knee and an equinus foot. We experienced a six-month-old girl with popliteal pterygium, in whom resection of abnormal fibrotic muscle belly, Achilles tendon legnthening, and Z-plastic lengthening of the skin resulted in partial correction of flexion deformity of the knee and equines contracture of the foot at one year follow-up.