Primary malignant bone tumours are rare neoplasms. However, osteosarcoma, chondrosarcoma and Ewing`s sarcoma are also included in this group of tumours. In the last 40 years, systemic chemotherapies coupled with surgical resections have provided significant improvements in survival. Surgery alone has a limited success, particularly in the treatment of osteosarcoma and Ewing`s sarcoma. Lately, the success of targeted treatment agents in the treatment of metastatic disease increases hopes.