Abstract: Objective To analyze the location, clinical characteristics, curative effect and prognosis of neuroendocrine carcinoma(NEC) in the digestive system. Methods Clinical data about 40 patients with NEC in the digestive system admitted to our hospital from January 1998 to December 2011 were retrospectively analyzed.The patients were followed up. Results NEC was located in esophagus of 14 patients and in stomach of 9 patients.Histological typing showed that 50% of the NEC was found to be small cell cancer.Twenty-six patients were treated with etoposide and cisplatin/carboplatin as the first line chemotherapy, with an effective rate of 23.1% and a disease control rate of 69.2%.The therapeutic effect was good for esophageal, gastric and intestinal NEC, whereas poor for pancreatic, hepatic and abdominal NEC.Twenty patients underwent combined irinotecan and fluorouracil treatment as the second line chemotherapy with an effective rate of 15% and a disease control rate of 65%.Of these 20 patients, 6 survived with a longest survival time of 47 months (mean 15 months). Conclusion NEC is frequently found in esophagus and stomach.Some NEC patients are sensitive to chemotherapy, but their remission and overall survival time are short.