Primary testicular lymphoma (PTL) is a rare disease accounting for 1% of non-Hodgkin's lymphoma. PTL occurs more frequently in older patients and is a potentially fatal disease. In the early stages (I and II), the treatment consists of orchidectomy followed by chemotherapy (CT) and prophylactic scrotal radiotherapy (RT) with/or without iliac and/or paraaortic lymph node RT. In the advanced stages (III and IV), CT is the treatment of choice whereas the place of scrotal RT is controverted. In both early and advanced disease intrathecal CT is warranted to prevent CNS relapse. New molecular approaches and/or more aggressive treatments are being explored.