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Background: Barrett's ulcer, which develops within Barrett's esophagus, is frequently responsible for bleeding. Perforation is a rare complication constituting a great challenge for diagnosis and management., Methods: Three personal cases and 31 published reports of perforated Barrett's ulcer were reviewed retrospectively. The site of perforation, clinical presentation, management, and outcome were assessed., Results: The clinical presentation proved to be heterogeneous and was determined by the site of perforation: this was the pleural cavity (20% of cases), mediastinum (20%), left atrium (16.6%), tracheobronchial tract (13.3%), aorta (13.3%), pericardium (10%), or pulmonary vein (6.6%). Early esophagectomy and esophageal diversion-exclusion were the most frequent procedures, and overall mortality was 45%., Conclusions: The poor prognosis of perforated Barrett's ulcer should be improved by earlier diagnosis and adequate emergent operation. Although early esophagectomy constitutes the recommended procedure, esophageal diversion-exclusion, which allows control of both sepsis and bleeding, is also of interest.