A 62-year-old man presented at our hospitalwith generalfatigue and tarry stool. Gastrointestinalendoscopy revealed a tumor in the first portion of the duodenum. Histologically, biopsy specimens indicated adenocarcinoma, and a subtotal stomach- preserving pancreatoduodenectomy was performed. The finalhistol ogicaldiagnosis was mixed adenoneuroendocrine carcinoma(MANEC)of the duodenum. Liver metastases appeared rapidly within 3 months after surgery. We report on the management of a rare case of MANEC in the duodenum.