Duodenal type follicular lymphoma is a rare malignancy accounting for less than 4% of primary non-Hodgkin lymphomas of the gastrointestinal tract and it is a new entity that was recently described in the new update WHO 2016. Data regarding long-term outcome are currently lacking, and for that reason, a consensus on the management of this disease has not been established and treatment. We report a case of a 57-year-old female patient diagnosed with duodenal- type follicular lymphoma grade 3a who was treated with R-CHOP. The aim of this study is to add more data for a greater characterization of the entity and thus select the best management for each case.