Primary adult Ewing's sarcoma is a rare entity. They most commonly occur in children and young adults. 6% of them are localized extraosseously. We present a case of a 51 year old patient with primary renal Ewing's sarcoma and multiple metastases in liver and iliac bone. Patients with metastatic disease are usually treated with aggressive chemotherapy and have a poor outcome. Our patient underwent complete surgical excision of tumour, and was treated with aggressive chemotherapy, respectively. Two and half years after presentation he is well, without any symptoms.