Summary Primary gastrointestinal (GI) lymphoma accounts for 30–50% of all extranodal non-Hodgkin’s lymphomas, making the GI tract the most common site of extranodal non-Hodgkin’s lymphomas. Most GI lymphomas belong to the B cell lineage. Burkitt lymphoma (BL) is a highly aggressive mature B cell neoplasm that occurs in three forms: endemic, sporadic, and immunodeficiency-associated. Sporadic BL accounts for 1–2% of all adult lymphomas and usually presents as an abdominal manifestation of extranodal disease involving the distal ileum or cecum. Primary BL of the duodenum is rare. However, this report emphasizes the importance of awareness of the malignancy potential of duodenal polyps. We report the case of a 70-year-old woman admitted to our ward with upper GI bleeding due to duodenal polyps. An upper GI endoscopic examination showed button-like polyps with central depression, and an immunohistochemical study of the polyps revealed a high-grade B cell malignancy (BL). Consequently, the patient was treated with aggressive chemotherapy. The tumors regressed after chemotherapy. Although primary duodenal Burkitt lymphoma is very rare, the possibility of malignancy should be considered if a patient presents with duodenal button-like polyps with a central depressed surface.