Two cases of pemphigus vulgaris in which oral lesions were the first signs of the disease are reported. The clinical signs and symptoms, histologic characteristics, and immunohistochemistry are discussed. Early recognition of oral lesions associated with the disease is of the utmost prognostic value. Treatment, which can only be symptomatic, usually consists of a combination of a corticosteroid and immunosuppressive medication. Because side effects may be serious, these medications should be prescribed and monitored by an experienced dermatologist.