Abstract: Objective:To characterize the clinical and epidemiological changes, treatments, and prognoses of primary esophageal small cell carcinoma (PESC). Methods:A retrospective analysis was conducted using the clinical epidemiology data of529 PESC patients se -lected from the clinical databases of 500 ,000 esophageal and gastric cardiac carcinomas of the Henan Key Laboratory for Esophageal Cancer Research (1992- 2015). Among these patients, 241 cases were included in the survival analysis. The five-year survival rate was calculated using Kaplan- Meier analysis, and the differences in survival rates were compared using the Log- rank analysis model. Re-sults: All529 PESC cases were analyzed, which accounted for 0. 2% of esophageal cancers diagnosed in the same period. The incidence of PESC increased annually (R2=0. 574 ). The survival rates for 1-, 2-, 3-, and 5- year of 241 PESC patients were 55%, 40%, 29%, and 9%, respectively, and the median survival time was 21. 9 months. On the basis of the VALSG criteria of lung small cell carcinoma, a statisti-cal difference was observed in the overall survival rates for limited and extensive diseases (P=0. 003 ), with the median survival time of 24. 3 and 17. 5 months, respectively. Furthermore, significant differences were observed on survival with various treatment modalities (P=0. 004 ). The median survival time of PESC patients treated with combined surgery and radiochemotherapies (28. 8 months) was lon-ger than those with either chemotherapy (17. 8 months,P=0. 015 ) or chemoradiotherapy (14. 5 months,P=0. 004 ). In limited disease pa-tients, the median survival time was longer in patients treated with surgery (27. 7 months) than in those without surgery (16. 2 months, P=0. 007 ). Notably, the biopsy diagnosis before surgery for PESC was only 40. 8%.Conclusion: PESC is a rare malignant carcinoma with increasing incidence. PESC presents poor prognosis, and the survival rate can be improved through combined therapies based on sur -gery. A high misdiagnosis rate for PESC is observed before surgery with biopsy.