BACKGROUND: Pemphigus vulgaris (PV) is an uncommon autoimmune disease characterized by blistering of the skin and mucous membranes. It has an approximate prevalence of 1 per 1,000,000 population, and is seen most often between 50 and 60 years of age. PV usually presents with oral lesions before affecting the skin. PV can be diagnosed by its clinical features, and by the characteristic histopathological features of acanthocytes inside suprabasal vesicles in the epidermis. PV used to be associated with a mortality rate of around 90% mortality before the introduction of corticosteroid therapy; advances in treatment have now brought the mortality rate down below twenty per cent. CASE REPORT: A 60-year-old female patient was referred with odynophagia for 2 months that had worsened over the past 10 days. She reported blisters in the mouth at the onset, apart from thrush, drooling, dysphagia, intermittent fever, and diarrhea. She felt her problems were related to her orthomolecular diet, and gave a history of gastric ulcers and genital herpes. Clinical examination showed mucosal hyperemia, apart from ulcers, thrush and white plaques in the buccal, gingival, palatal and hypopharyngeal mucosae. She was prescribed amoxicillin-clavulanate, fluconazole, and nystatin without improvement. Initial laboratory test results were as follows: Hemoglobin = 15 g/dL; TLC = 13,800 / µL, with 3% basophils, 19% lymphocytes and 0% atypical lymphocytes; Creatinine = 1.3 mg/dL; Glucose 104 mg / dL; PCR +, HIV - VDRL - FAN - FR negative. The following conditions were considered in the differential diagnosis: oral candidiasis, pemphigus vulgaris, pemphigoid, Stevens-Johnson syndrome, paraneoplastic pemphigus, Behçet's disease, Chron. EDA and mild antral gastritis. Biopsy of lesions showed squamous epithelium with suprabasal blisters, confirming the diagnosis of pemphigus vulgaris. The patient is now in remission, controlled with prednisone and dapsone. CONCLUSION: Although uncommon, PV is associated with a high mortality rate if not diagnosed and treated properly. This report work highlights the importance of early diagnosis by otorhinolaryngologists, as the first manifestation of the disease are usually oral.