A 75-year-old man with a complaint of right hypochondrial pain consulted our hospital, and was diagnosed as having acute cholecystitis. We performed percutaneous transhepatic gallbladder drainage (PTGBD), and the cholecystitis resolved. However, obstructive jaundice occurred 2 weeks later. Thus, we performed an in-depth investigation and detected duodenal papilla cancer (cT3N1M0, Stage III). Pancreatoduodenectomy was performed. As postoperative histological diagnosis yielded positive and strongly positive immunostaining for synaptophysin and Ki-67, respectively, we diagnosed the patient as having neuroendocrine carcinoma (NEC) of the duodenal papilla. Three months after surgery, computed tomography (CT) scan showed multiple liver metastases and lymph node metastasis. Chemotherapy with carboplatin and etoposide was administered, but severe neutropenia developed, and therefore, the chemotherapy was discontinued. Subsequently, we decided on a policy of best supportive care (BSC). The patient died 11 months after surgery. NEC of the duodenal papilla is reported to be a rare and rapidly progressing disease and is associated with a very poor prognosis. Herein, we report a case of a patient in whom NEC of the duodenal papilla was resected.