Primary small-cell carcinoma of the esophagus is an uncommon esophageal malignancy. This report details the clinical and pathologic aspects of 11 cases seen at our institution over 20 years, as well as 123 other cases reported in the literature. Small-cell carcinomas of the esophagus show considerable histologic heterogeneity. Neurosecretory granules can be found in the majority of cases and some show evidence of multidifferentiation. Like primary small-cell cancers of the lung, those in the esophagus are highly aggressive, are usually associated with spread at the time of diagnosis, and have a dismal prognosis regardless of treatment. The possible origin of this interesting variety of esophageal neoplasm is also discussed.