Popliteal pterygium syndrome (PPS) is a rare autosomal dominant disorder consisting of popliteal webs and craniofacial, genitourinary, and extremity anomalies. The popliteal webs make this syndrome particularly important to the orthopaedic surgeon. The resultant fixed flexion deformity can be severe, at times approximating the ischium to the calcaneus. The neurovascular bundle is contained in the web's posterior subcutaneous (S.C.) margin, which makes surgery precarious and difficult. We reviewed four patients between 1964 and 1986, three of whom were severely affected with fixed flexion deformities at the knee. Distal femoral shortening and extension osteotomies provided good results, and this procedure should be considered earlier rather than later in treatment of this problem.