Neuroendocrine carcinoma rarely occurs in the duodenum, and most cases of neuroendocrine carcinoma in the duodenum show rapid progression of the disease. Such cases have poor prognosis even with radical surgery with or without chemotherapy with low 5-year survival rate. We present a case of a 52-year-old man who presented with abdominal pain of 1-month duration and one episode of vomiting. Upper gastrointestinal endoscopy revealed polypoidal lesions in the first and second part of the duodenum. Whipple's procedure was performed. Diagnosis of poorly differentiated neuroendocrine carcinoma was made with extension to pancreas with peripancreatic lymph node metastases. The patient expired on post operative day 17 following cardiac arrest.