Primary small cell carcinoma (SCC) is a rare entity in the esophagus, although it is relatively common in the lung. Similar to small cell lung carcinoma, SCC of the esophagus is aggressive and the prognosis is poor. In this article, we report 2 patients with primary SCC of the esophagus with distal metastasis. They received chemotherapy and radiotherapy, and survived for 6 months and 10 months, respectively.