RESUMEN
Fibrocartilaginous dysplasia (FCD) has occasionally led to a misdiagnosis and wrong decision which can significantly alter the outcome of the patients. A 9-yr-old boy presented with pain on his left distal thigh for 6 months without any trauma history. Initial radiographs showed moth eaten both osteolytic and osteosclerotic lesions and biopsy findings showed that the lesion revealed many irregular shaped and sclerotic mature and immature bony trabeculae. Initial diagnostic suggestions were varied from the conventional osteosarcoma to low grade central osteosarcoma or benign intramedullary bone forming lesion, but close observation was done. This study demonstrated a case of unusual fibrocartilaginous intramedullary bone forming tumor mimicking osteosarcoma, so that possible misdiagnosis might be made and unnecessary extensive surgical treatment could be performed. In conclusion, the role of orthopaedic oncologist as a decision maker is very important when the diagnosis is uncertain.