Duodenal-type follicular lymphoma (D-FL) is a special type of follicular lymphoma, which tends to occur in the descending segment of the duodenum. The lesion is mostly limited to the mucosal layer. The treatment approach for D-FL has not been clearly established and the watch and wait (WW) approach is generally recommended as a major option. Since D-FL may be transformed into a more serious type of lymphoma, it is of clinical significance to explore active treatment methods. We diagnosed and successfully treated a case of D-FL with Endoscopic submucosal dissection (ESD). Because D-FL is limited to mucosa in the descending segment of the duodenum, ESD can completely dissect the lesion to achieve the purpose of complete resection. Compared with the WW, the method of WW after endoscopic therapy is more active, safe and effective.