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Primary carcinoma of the duodenum, especially mucinous carcinoma of the duodenum, is extremely rare. We present a case of a long-term response to chemotherapy in mucinous carcinoma of the duodenum with multiple distant metastases. A 60-year-old man was admitted to our hospital with epigastric pain and jaundice. CT showed a thickening of the duodenal wall; extensive lymphadenopathy around the head of the pancreas, in the para-aortic region and the mediastinum; suspected peritoneal dissemination; lung metastases; and bone metastases. An upper gastrointestinal endoscopy revealed a duodenal stenosis in the descending limb with irregular mucosa, and a diagnosis of mucinous carcinoma of the duodenum was made on the basis of the histological analysis of the biopsy sample. Double bypass surgery involving a choledochojejunostomy and gastrojejunostomy were performed for obstruction of the duodenum and common bile duct. After FOLFOXIRI therapy was initiated, the tumors were reduced markedly. Despite withdrawal after 28 courses of chemotherapy, the patient achieved a long-term response for 10 years after the initiation of chemotherapy.