Materials and methods: En bloc resection was performed in 13 patients with bone tumors between October 1991 and December 2010 in our clinic. The mean age of the patients was 19.46 years (range 7–42 years) at the time of surgery. Histological diagnosis was osteosarcoma in seven cases, Ewing's sarcoma in three cases, giant cell tumor in one case, osteoblastoma in one case and fibrous dysplasia in one case. In all cases either the femur or tibia was involved. Aim: The purpose of this retrospective study was to report the long-term follow-up results of the reconstruction of bony defects with Ilizarov distraction osteogenesis using the bone transport method following en bloc resection of bone tumors. Results: The average follow-up period was 157.23 months (range 32–288 months), and the bone defect after resection was 14.61 cm ± (9–24 cm). The mean Musculoskeletal Tumor Society score of the patients was 89.46 (83–96) at the final follow-up. The mean Knee Society Scale scores of patients in whom reconstruction was performed around the knee joint were 74.3 (51–84). The mean foot and ankle disability index of patients with a tumor around the ankle joint was 81 (73–95). Quality of life of the patients according to the SF-36 and BQUILI indexes was scored as 104 (88–150) and 4 (0–13), respectively. Conclusion: From the long-term follow-up results, reconstruction with distraction osteogenesis seems to be an efficient method in patients with long life expectancies. However, a long external fixation time is a disadvantage of this technique. Problems in patient compliance and possible complications such as nonunion should be managed promptly.