Small cell carcinoma of the esophagus (SCEC) is a rare tumor with its particular biologic features, distinct from squamous and glandular carcinoma of esophagus. Although initial symptoms can be similar (with metastatic dissemination and paraneoplastic syndromes at presentation more frequent in SCEC), differences in age and sex distribution, tumor location, radiological and endoscopic findings, clinical course and prognosis have been observed between SCEC and other esophageal malignancies. SCEC should be considered a systemic disease, and by analogy to bronchogenic small cell carcinoma, multimodality approach including chemotherapy is recommended. In patients with limited disease, irradiation or surgery should be offered after induction chemotherapy to manage local disease. However, optimal treatment schedule has not yet been defined. In the future, registration of all SCEC cases and careful analysis of prognostic factors in the larger multi-institutional series could contribute to further progress in treatment outcome.