We present the case of a 63-year-old woman with herpetiform pemphigus and disseminated esophageal carcinoma which began to manifest themselves simultaneously. Some cases of herpetiform pemphigus associated with neoplasms have previously been reported, but these have primarily been bronchopulmonary. The association of any type of pemphigus with esophageal cancer is very infrequent. We should be alert to the infrequent varieties of pemphigus and atypical bullous diseases, because they sometimes do not meet the diagnostic criteria of paraneoplastic pemphigus, but are the first manifestation of an underlying neoplasm.