Primary small cell carcinoma of the esophagus (SmCC) is an uncommon aggressive tumor characterized by early systemic dissemination and poor prognosis, regardless of the methods of treatment. The optimal treatment strategy remains uncertain. A retrospective study was conducted to review the results of non-operative treatment for patients with limited and metastatic esophageal SmCC. Between 1993 and 2003, 10 patients were diagnosed to have primary esophageal SmCC in our institution. Six of them had disseminated diseases, whereas the other four had limited disease upon diagnosis. All patients were managed non-operatively by either chemotherapy and/or radiotherapy. The overall median survival was 8 months (range, 2-62 months). The survival was 4-62 months for patients with limited disease, whereas it was 2-10 months for patients with disseminated disease at initial diagnosis. In summary, the current study demonstrated satisfactory palliation could be achieved with chemo-radiation for patients with limited disease; however, the ultimate role of primary chemo-radiation for esophageal SmCC must await results from randomized trials.