Primary non-Hodgkin's lymphoma of the testis (PTL) accounts for about 9% of testicular neoplasms and 1-2% of all non-Hodgkin's lymphomas. It is the most common testicular malignancy in elder men. Anecdotal reports associated PTL development with trauma, chronic orchitis, cryptorchidism, or filariasis exist, but no case-control studies have confirmed their etiologic significance. Diffuse large B-cell lymphoma (DLBCL) is the most common histotype in primary forms; aggressive histologies, especially Burkitt's lymphoma, are prevalent in cases of secondary involvement of testis. The most common clinical presentation is a unilateral painless scrotal swelling, sometimes with sharp scrotal pain or hydrocele. Systemic B symptoms are present in 25-41% of patients with advanced stage. Less frequently, abdominal pain, and ascites can be seen in patients with involvement of retroperitoneal lymph nodes. Bilateral testicular involvement is detected in up to 35% of patients. Although good results with doxorubicin-containing chemotherapy, followed or not by radiotherapy, have been reported, a high proportion of patients with stage I-II diseases experience aggressive relapses, and patients with advanced disease have a very poor prognosis. PTL has a propensity to disseminate to other extranodal organs, including the controlateral testis, CNS, skin, Waldeyer's ring, lung, pleura, and soft tissue. Orchidectomy followed by R-CHOP combination, with CNS prophylaxis, and prophylactic irradiation of the contralateral testis is the recommended first-line treatment for patients with limited disease. Management of patients with advanced or relapsed disease should follow the worldwide recommendations for nodal DLBCL.