Autoimmune blistering diseases are a rare group of mucocutaneous disorders that can result in irreversible sequelae and death if accurate diagnosis and treatment are not rendered promptly. Pemphigus vulgaris and bullous pemphigoid are the two most common diseases in this group, which are disorders characterized by the production of autoantibodies that target structural proteins important for the maintenance of intercellular and cell-to-basement membrane adhesion. Before the advent of corticosteroid therapy, pemphigus was fatal, with a mortality rate of up to 75% in the first year. It is still a serious disorder, but the 5% to 10% mortality rate is due to the side effects of the therapy. In 75% to 80% of cases, PV lesions first appear in the oral cavity. Dentists are, therefore, in a unique position to recognize the oral manifestations of the disease, allowing early diagnosis and initiation of treatment. The diagnosis is based on clinicopathological examination and immunofluorescence testing. Systemic corticosteroids and steroid-sparing agents are the mainstays of treatment. Topical corticosteroids may also be used to accelerate the healing of persistent oral lesions. This article describes a 55-year-old woman with multiple chronic ulcers in the oral cavity, in which pemphigus vulgaris was diagnosed two months after the symptoms first appeared. This article also reviews the current literature on the diagnosis and treatment of the condition.