We report a complicated case of osteosarcoma of the proximal tibia. A 15-year-old boy was referred to us and underwent distraction osteogenesis reconstruction. We administered preoperative chemotherapy for five cycles. Clinical response was determined to be complete by radiography. Marginal excision was then performed with preservation of the proximal tibial epiphysis. Metaphyseal reconstruction (type 2) was performed with distraction osteogenesis. Postoperative chemotherapy also was administered for five cycles. Two years later, the patient developed a deep infection. He underwent curettage and a pedicle peroneal flap transfer, which did not cure the infection. Infected tissues were excised, and shortening-distraction was carried out with the Ilizarov frame. The infection was cured; however, a leg length discrepancy and deformity resulted from frame instability. Four years after the initial operation, the patient fractured his reconstructed leg in a traffic accident. We performed osteosynthesis, deformity correction, and lengthening with the Ilizarov method. We were able to correct the defects using distraction osteogenesis, eventually restoring normal function. Epiphyseal preservation and reconstruction by distraction osteogenesis can provide an excellent outcome, resulting in a stable reconstruction that functionally restores the native limb. Distraction osteogenesis avoids some complications but may involve others, which require detection and appropriate management.