An extremely rare case of pemphigus vulgaris in a 6-year-old girl with prominent oral manifestations is presented. The disease was first noted at the age of 2 years, yet no diagnosis was made. Clinical, histopathologic, and immunologic criteria served to confirm the diagnosis. A review of the literature has revealed 22 well-documented cases of pemphigus vulgaris in children under the age of 17 years. It is concluded that although oral pemphigus vulgaris is rare in young persons, it must be included in the differential diagnosis in those cases that have a prolonged history of erosive or bullous lesions.