The aim of this paper was to analyze the prognostic features, clinical presentation, diagnosis and treatment of primary renal sarcomas. A thorough review of the literature was made using the PubMed database. Sarcomas of the kidney are very rare malignant tumors, with a very poor prognosis compared with the other urogenital sarcomas. Several histological types have been reported, with the most common type being leiomyosarcoma. Besides complete surgical resection with wide margins, anatomical, histological, molecular and genetic factors should be taken into consideration regarding the prognosis
