Primary testicular non-Hodgkin's lymphoma (TNHL) was first described as a clinical entity in 1866. It is a rare disease which accounts for 1% of all non-Hodgkin's lymphoma, 2% of all extranodal lymphomas and 5% of all testicular neoplasms. Hemophagocytic syndrome (HPS) is a rare but distinct condition caused by inappropriate and dysregulated activation of the immune system. We report a 47-year-old man with primary TNHL who developed HPS four months after occurrence of scrotal swelling. To the best of our knowledge, primary TNHL associated with HPS has not previously been reported.