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Pemphigus vulgaris (PV) is a chronic autoimmune mucocutaneous disease. In most cases, the initial manifestation occurs in the mouth as multiple ulcerations preceded by blisters that rupture and later spread to other mucous membranes and the skin. Esophageal impairment is rare. We report a case of PV with esophageal involvement in a 53-year-old woman who sought medical care, complaining of diffuse painful lesions in the oral cavity for approximately 1 month, with no improvement with nystatin. Upper digestive endoscopy (UDE) was performed with findings of vesiculobullous lesions in the proximal and middle esophagus, which were biopsied and for which histopathology confirmed PV in the esophageal mucosa. We draw attention to UDE as important for the diagnosis, and it should be indicated in patients with vesiculobullous lesions of the mouth, especially if there are esophageal complaints.