Abstract： Objective To investigate prognosis factors of primary small cell carcinoma of the esophagus （PSCCE）, and to optimize the treatment strategy of PSCCE. Method We retrospectively analyzed clinical data of 15 patients （13 males and 2 females with an age of 57.7±2.3 years） with middle thoracic PSCCE in West China Hospital from June 2005 to February 2010. We searched ISI and MEDLINE from April 2001 to February 2010 to extract clinical data of 139 PSCCE patients with 94 males and 45 females with an age of 63.3±10.7 years. We analyzed prognosis factors of the 139 patients including gender, age, tumor location, pathological type, lesions stage and treatment strategy by Kaplan-Meier. Difference in survival curves between limited disease patients and extended disease patients was tested by log-rank test. Results Among the 15 patients, 14 patients had limited disease, and 1 patient had extended disease. Their data were not included in survival analysis because the follow-up was incomplete. Among the 139 patients, 88 patients had limited disease with their 2-year survival rate of 31.8% （28/88）. Fifty-one patients had extended disease with their 2-year survival rate of 7.8% （4/51）. The 2-year survival rate between limited-disease patients and extended-disease patients was statistically different（P＜0.05）. Radiation therapy in combination with chemotherapy had significant influence on the survival rate of patients with either local lesions or advanced lesions（P＜ 0.05）, while other factors such as gender, age and tumor location had no significant influence on their survival rate（P＞0.05）. Conclusion Chemotherapy is the fundamental treatment of PSCCE, which plays an important role in reducing PSCCE preoperative staging and restraining PSCCE postoperative recurrence and metastasis. Surgery and radiation therapy are effective for patients with local lesions. Local treatment in combination with chemotherapy is effective for patients with limited disease. Radiation therapy in combination with chemotherapy is the standard therapy for patients with extended lesions,