Abstract: Objective To analyze the location,clinical characteristics,curative effect and prognosis of extra-pulmonary neuroendocrine carcinoma. Methods Clinical data about 76 patients with extra-pulmonary neuroendocrine carcinoma admitted to our department in 1998-2011 were retrospectively analyzed. Results Extra-pulmonary neuroendocrine carcinoma occurred mainly in digestive system and mediastinum.The total effective rate,disease control rate and MTTP of first-line treatment for extra-pulmonary neuroendocrine carcinoma were 31.37%,72.55% and 3.7 months,respectively.The total effective rate,disease control rate,MTTP and MST of second-line treatment for extra-pulmonary neuroendocrine carcinoma were 13.33%,46.67%,1.6 months and 15.2 months,respectively. Conclusion Certain neuroendocrine carcinomas are relatively sensitive to chemotherapy.However,the remission time and total survival time of patients with a higher malignancy are short.