BACKGROUND : Because the duodenum is fixed onto the retroperitoneum, duodenal intussusception is usually impossible except in cases of malrotational abnormality. Although cases of duodenal intussusception without malrotational abnormalities have been reported, it is unclear whether they constitute true intussusception or simple mucosal prolapse. METHODS : A 66-year-old woman presented with whole-body edema and malaise. Blood analysis indicated severe anemia and cholestasis. Endoscopic examination revealed a pedunculate polyp on the second part of the duodenum that migrated distally with mucosal elongation. Computed tomography showed duodenal intussusception. A tumor as the lead point and retroperitoneal structure, including the head of the pancreas and fat, invaginated beyond the duodenojejunal flexure. She was diagnosed with ampullary adenoma caused repeated intussusception that reduced spontaneously and underwent pancreaticoduodenectomy. Laparotomy showed tumor prolapse beyond the duodenojejunal flexure without intussusception. There was no evidence of malrotational abnormality. She was discharged with no complications. CONCLUSIONS : We report true duodenal intussusception without malrotational abnormality. This phenomenon was also associated with mucosal prolapse.