Stage IV disseminated non-Hodgkin lymphomas show kidney involvement with frequency; on the contrary, primary kidney lymphoma, as the sole presenting feature, is a very rare disease. Non-Hodgkin histological pattern, atypical symptoms, marked discrepancy between parenchymal involvement and impairment of renal function are the main features of this tumor. A case-report of a primary kidney non-Hodgkin lymphoma will be described; a short review of literature on this subject will be discussed.