We conducted a retrospective tumor registry review of a 36-year experience in a university center and identified 10 patients with duodenal lymphoma (five localized, five disseminated). Histologic types included diffuse large B-cell in four patients, mucosa-associated lymphoid tumor in three, and Hodgkin, follicular, and unclassified (one each). Treatments included chemotherapy in four patients, radiation therapy (RT) in two patients, Helicobacter pylori treatment in two, and observation in one. Five patients underwent operations (emergent in two, elective in three) for indications including massive bleeding in two patients, obstruction in two, or both in one. Survival for surgical group was 25 per cent at 1 year. One-year survival for nonsurgical group was 100 per cent, and all nonoperated patients lived at least 5 years, except for one who is alive 2 years after diagnosis. Surgical patients were younger and had more advanced lesions and less favorable cell types. When operation is required for bleeding or obstruction from secondary tumors in younger patients with disseminated disease, surgical challenges are formidable and survival is very limited. Tumors of less aggressive histology have far better prognosis.