Ewing`s sarcoma –a member of round cell tumors– is a primary malignant bone tumor that usually localises in the diaphysis of long bones and can be seen mostly between 5 and 25 years of age. Ewing`s Sarcoma is the second most frequent malignant bone tumor and can also occur in the soft tissue. In some cases tumor shows the characteristics of neural origin and is also named as primitive neuroectodermel tumor (PNET). Both tumors are accepted as similar tumors. In 90% of the cases t(11; 22)(q24; q12), and in 5–10% of the cases t(21; 22)(q2; q12) abnormalities are detected. Osteomyelitis, lymphoma, mesenchymal chondrosarcoma, small cell osteosarcoma, metastatic neuroblastoma, small cell carcinoma of the lung, embriological rhabdomysarcoma and eosinophylic granuloma should be taken into consideration for differential diagnosis. Ewing`s sarcoma tends to metastasize primarily to lung. Besides the general sarcoma staging system I-II-III A, B), a unique staging system is also used for Ewing`s sarcoma. In this system in EW-I the sarcoma is localised to bone, in EWII the tumor extends to soft tissue, in EW-III there is multiple bone involvement and in EW-IV there are distant organ metastasis. CT, PET-CT, bone scan and MR are utilized for the detection of the metastasis. Ewing`s sarcoma is usually sensitive to chemotherapy and radiotherapy. Besides these, surgical treatment has shown to have positive effect to the local control of the tumor and prognosis. VACA (vincristine, actinomycin-D, cyclophosohamide and adriamycine), VAIA (vincristine, actinomycine-D, ifosfamide, adriamycine) or EVAIA (VAIA+etoposide) are the most common chemotherapy protocols. Chemotherapy with surgery is the primarily accepted treatment system for Ewing`s Sarcoma. Up to date investigations revealed that the survival rate of Ewing`s sarcoma may rise up to 80%.