Small-cell carcinoma of the esophagus is a rare tumor and has received little attention until recent years. It should be differentiated from the far more common poorly differentiated squamous-cell carcinoma of the esophagus, because treatment by surgical resection alone or by radiation therapy results in limited survival of a few months. It is now recognized that esophageal small-cell carcinoma presents with early widespread dissemination and is chemosensitive, similar to primary small-cell carcinoma of the lung. We report on a patient with small-cell carcinoma of the esophagus treated with combination chemotherapy consisting of cyclophosphamide, vincristine, and VP-16 followed by local radiation therapy. Pathologic complete remission was achieved. The patient is currently in remission 22 months after diagnosis, the longest survival reported thus far.