Polypoid vascular and lymphatic malformation of the small intestine is rare. We report the case of a polypoid vascular and lymphatic malformation of the duodenum in a 58-year-old woman presenting with post-prandial epigastric discomfort. She did not have common symptoms of intestinal vascular and lymphatic malformation, such as gastrointestinal bleeding, steatorrhea, and hypoalbuminemia. A 5.0x0.8-cm semipedunculated polypoid lesion was found in the duodenal bulb with protrusion into the prepyloric antrum and was successfully removed using an endoscopic snare.