Brunner's gland adenoma is an extremely rare benign small bowel neoplasm, often discovered incidentally during upper gastrointestinal endoscopy or radiological diagnostics. In few cases, it tends to cause gastrointestinal hemorrhage or intestinal obstruction. We report here our experience with a 47-year-old woman with a Brunner's gland adenoma of more than 6 cm in size, located in the first part of the duodenum and causing gastrointestinal bleeding. Initially, we performed a partial endoscopic resection using endoloop and snare alternatively to prevent severe bleeding. A rest endoscopic polypectomy with the submucosal dissection technique was planned. However, on request of the patient, an elective surgical duodenotomy with submucosal resection of the remaining small duodenal tumor was performed. To better define the patient's characteristics and treatment options of such lesions, we performed a systematic review of the available literature in PubMed. Recently, an endoscopic removal is being increasingly practiced and is considered as a safe treatment modality of such lesions.