A case of pemphigus vulgaris with esophageal involvement is discussed and 10 other reported cases are reviewed. Esophageal involvement is a rare but serious occurrence. It is most readily diagnosed by endoscopy when dysphagia or odynophagia in a patient with pemphigus vulgaris or with a history of pemphigus vulgaris does not respond to appropriate adjustment of corticosteroid or other therapy. All patients with esophageal pemphigus vulgaris were middle-aged women. None had skin lesions at the time esophageal disease was diagnosed, and five patients appeared to be in complete remission, with neither skin nor oral lesions, when esophageal involvement was discovered.