The association of adenocarcinoma with Barrett's esophagus stimulated a review of our experience to study the clinical presentation, pathology, and results of management. Nineteen patients (15 men and four women) satisfied the criteria of primary adenocarcinoma arising in columnar epithelium in the esophagus. The majority had dysphagia (95%) and weight loss (63%). Nearly three fourths of the patients also had a history of hiatus hernia or esophagitis. Diagnosis was confirmed preoperatively in all by endoscopic biopsy and/or cytologic study. Potentially curative resection was performed in 15 patients and palliative procedures in four. Fourteen patients had advanced (Stage III) disease and only five had Stage I or II disease. Multicentric disease within the esophagus was found in seven patients. Postoperative complications included empyema, hemothorax, and pneumonia (one case each). The only postoperative death resulted from complications of previously undetected brain metastases. The median survival of the 15 patients having resection for cure is 12 months. Four are alive, one with disease at 46 months and three free of disease at 19, 87, and 93 months. All four patients undergoing palliative procedures died within 8 months. The study demonstrates that multifocal presentation of the tumor is common in this group of patients and that long-term survival is possible when early tumors are managed aggressively.