Duodenal follicular lymphoma (DFL) is a rare variety of non-Hodgkin's lymphoma of the gastrointestinal tract that usually carries a favorable course, recognized as a new entity in 2016. It is usually diagnosed at an early stage located predominantly in the second portion of the duodenum. We report the case of a 74-year-old male patient with epigastric pain in whom gastroscopy revealed white mucosal nodules that were pathologically diagnosed as grade 1-2 DFL. Staging investigations revealed secondary lesions in the spleen and at the base of the tongue together with latero-cervical adenopathy. The tumor was stage IV according to the Lugano staging system. We reviewed the recent (last five years) literature defining the importance of combination therapy in the advanced stage. The patient achieved complete remission of the disease through chemoimmunotherapy following the Rituximab-Bendamustine scheme.
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