Small cell carcinoma of the esophagus is a rare and aggressive tumor with early widespread dissemination. In this retrospective study, we report epidemiologic, histologic, and clinical characteristics of small cell carcinoma of the esophagus from the analysis of 10 patients, with a literature review. Between 1993 and 1998, 10 patients with small cell carcinoma of the esophagus were treated in our institution, representing 2.8% of all esophageal malignancies diagnosed during this period. Four patients sought treatment for limited disease, whereas six patients had distant metastasis at the time of diagnosis. All patients received polychemotherapy, and a complete response was observed in eight patients. Seven of these patients received subsequent locoregional radiotherapy, with endoesophageal brachytherapy in two patients. The overall median survival was 15.5 months (range, 2–36 months) for all of the patients. In limited stages, the overall median survival was 18.5 months (range, 2–36 months), whereas it was 11 months (range, 6–19 months) for the extensive stage at initial diagnosis. In this article, we report our experience with this uncommon neoplasia and attempt to make comparisons with the cases published in the literature regarding location, symptoms, histopathologic diagnosis, and treatment. We conclude that the optimum treatment seems to be the same as for small cell carcinomas of the lung, that is, a multidrug combination chemotherapy regimen used alone or with sequential radiation.