Testicular lymphoma is a lethal disease with a median survival of approximately 12 to 24 months. It is the most common testicular malignancy in men older than 60 years of age. Testicular lymphoma has a predilection for widespread dissemination to unusual sites, including the central nervous system, contralateral testis, Waldeyer's ring, skin, and lung. Doxorubicin based chemotherapy with prophylactic intrathecal chemotherapy and radiation to the contralateral testis seems most promising. This review article will focus on the presentation, pathology, patterns of relapse and challenges in improving the outcome of this disease.