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The authors report a case of primary adenocarcinoma of the second portion of duodenum in a 56-years-old man radically managed by pancreaticoduodenectomy. They remark the rarity of this pathology, the difficulty of making diagnosis peculiar to neoplasms of the small intestine and the difficulty of treatment peculiar to tumours of duodenum. The possible therapy is surgical. Radio and chemiotherapy don’t significantly improve survival. Better results can be obtained only with an early diagnosis.