Bone sarcomas are extremely rare representing approximately 0.2% among all cancer types. Due to the rarity of these tumors both the patients and the physicians can overlook or misinterpret the first often unspecific symptoms of these tumors (pain, limping and swelling). Therefore, radiographic examination (X-ray and/or MRI) of the involved region in case of inexplicable symptoms is strongly recommended. The most common primary malignant bone tumor entity is osteosarcoma, followed by chondrosarcoma and Ewing sarcoma. Osteosarcomas and Ewing sarcomas occur predominantly in children, adolescents and young adults, while chondrosarcomas primarily affect older patients. Most of the tumors are located in the extremities and the pelvis and in about 90% of cases the surgical treatment can be performed by means of a limb-sparing wide resection. An endoprosthetic or biological reconstruction of the resulting defect, depending on several patient- und tumor-related factors, usually is necessary. Apart from the surgical treatment, patients with osteosarcoma and Ewing sarcoma require a pre- and postoperative chemotherapy, while Ewing sarcoma patients often undergo radiation therapy as well - in some cases as the only local therapy option. Regular follow-up examinations are required after the completion of treatment for the early detection and management of local and/or systemic recurrences. Diagnosis and therapy of these rare tumors must be obtained by an interdisciplinary approach for optimal patient care (i.e. oncologist, radiologists, pathologists, orthopaedic surgeons, radiotherapists). Without an interdisciplinary proceeding the risk of mistakes increases significantly, which can have grave consequences on the patients' prognosis and functional outcome. Therefore, the centralized treatment of these patients in specialized sarcoma centres is recommended. Georg Thieme Verlag KG Stuttgart · New York.