A case of popliteal pterygium syndrome is presented and the various possibilities of treatment so far reported in the literature are discussed. The medical history of the 22-year-old male patient shows that there is no uniform concept in its therapy and that even multiple operations, which dealt only with the skin contracture and the shortened achilles tendon, were not successful. An extensive neurolysis of the sciatic nerve, its transposition into deeper muscle layers and slow extension of the knee joint with a dynamic splint did improve the patient's condition and this seems to be the concept to follow even in patients with multiple previous operations. For children with popliteal pterygium syndrome, an evaluation of the severity of contracture in degrees would be useful and a conservative treatment with splints should be begun within the first months after birth. In case of no response to conservative therapy, an operation becomes necessary. Surgery consists of the transposition of the sciatic nerve into deeper layers with simultaneous excision of fibrotic tissue, flap transfer or Z-plasty and postoperative extension of soft tissue in the knee by splinting and physiotherapy.