We report a 5-year clinical follow-up and the operative details of a patient who was born with popliteal pterygium and ipsilateral upper amelia. There were no craniofacial, genitourinary, or other extremity abnormalities, other than severe pes equinovarus. At age 3 weeks, excision of the pterygium and partial release of the hind foot had accomplished a significant but incomplete release of the knee contracture. At age 11 months, a second release of the pterygium, combined with complete reconstruction of the foot, accomplished a straight limb. Presently at the age of 5, the boy has 40 degrees limitation to full extension at the knee but walks, runs, and behaves like all the other children in the nursery school. This combination of congenital malformations and early surgical intervention has never been described in the orthopedic literature.