Follicular lymphoma (FL) is one of the most common types of non-Hodgkin lymphoma (NHL), and the gastrointestinal tract is the most commonly involved ex-tranodal site. Primary duodenal FL (DFL), which mainly involves the second portion of the duodenum and has an excellent prognosis, is an unusual entity with only a few reported cases in the literature. The first reported case of primary duodenal follicular lymphoma was in 1997, and we could not find similar case reports in Taiwan. We report the case of a 70-year-old woman who underwent esophagogastroduodenosco-py due to mild, persistent epigastric pain. Endoscopic findings revealed numerous whitish and small submucosal nodules scattering diffusely in the second and third portions of the duodenum. The biopsy of these lesions revealed a pathological result consistent with FL. This elder patient with gastrointestinal symptoms was referred to the Oncology department for further management, and radiation therapy was chosen for treatment. Neither lesion aggregation nor lymphadenopathy was noted during the 4-year follow-up period. In conclusion, this was an uncommon case of DFL re-sponding favorably to radiation therapy and having an excellent prognosis. However, due to the lack of cases reported in Taiwan, further studies are needed to clarify the disease’s characteristics, therapeutic approach, and prognosis.