Biopsy followed by tumour removal and replacement by autologous bone tumour at the distal end of the femur were performed. Histologically, the tumour proved to be benign. Three years later progression was observed and epiphyseodiaphyseal resection was made because of on excessive tumour. Histology revealed a fibrosarcoma. Consolidation was hindered by osteomyelitis and fracture, recovery could be achieved only by a series of operations. These histological and operative difficulties are documented and the importance of extremity-saving operations is discussed. Our patient has been free of complaints 9 years after epiphyseodiaphyseal reseaction.