Testicular lymphoma is a rare and deadly disease representing 1% to 2% of all non-Hodgkin lymphomas (NHLs) and approximately 5% of all testicular neoplasms. Our objective is this study was to identify the presenting signs and symptoms, treatment and outcome of patients with testicular lymphoma diagnosed at our institution from 1992 to 2005, and to identify any differences in survival based on Ann Arbor Stage and International Prognostic Index (IPI). A retrospective chart review was performed to identify demographic characteristics, presenting signs and symptoms, treatment and outcomes. Survival was assessed using Kaplan-Meier survival curves and log-rank testing. Thirteen cases were identified; 1 of these cases was ultimately excluded due to a diagnosis of lymphoma a year before he presented with testicular involvement. Of the remaining 12 cases, the mean age was 65 years. Most patients presented with testicular and scrotal swelling or mass. B symptoms (weight loss, fever, night sweats) were present in 1 case. Of the 12 cases, 7 cases were stage I, 1 stage II and 4 stage IV. International Prognostic Index was low risk (</=1) in 7 cases and high risk (>1) in 4 cases. Orchiectomy was performed in all cases. Three patients received no further treatment. Of the remaining 9 patients, overall 7 received systemic chemotherapy, and 7 radiation therapy (prophylactic in 6). Three patients received intrathecal chemotheraphy prophylaxis. Seven patients achieved complete remission. Four patients (57%) relapsed following complete remission. Median time to relapse was 32 months (range 11 to 73 months). Six patients died. Median survival was 29 months, and was significantly different between early versus advanced stage (stage I/II disease: 71 months; stage IV: 5 months p = 0.007). Testicular lymphoma is a rare and deadly form of extra-nodal lymphoma. Survival was significantly different in early stage I/II and IPI low-risk versus advanced stage IV and IPI high-risk disease. Randomized, prospective treatment trials may help to establish better treatment strategies.