A 10-year-old boy with vesicles and eroded lesions on his labia, oral mucosa, and trunk was diagnosed as having pemphigus vulgaris. The diagnosis was confirmed by histopathologic and immunofluorescence studies. The patient was successfully treated systemically with methylprednisolone and azathioprine. After 4.5 years the therapy was discontinued and the patient has had no relapse over the last 4 years.