Juvenile pemphigus vulgaris (JPV) is a rare variant of pemphigus vulgaris (PV) occurring in childhood and adolescence that has similar symptoms and the same histological and immunopathological features as classic adult PV. A 14-year-old boy presented with multiple erosions on the oral mucosa and multiple bullous skin lesions that had appeared 4 months earlier. He reported that the oral lesions appeared suddenly, before the skin lesions. Histological examination revealed the characteristic features of pemphigus vulgaris, with a suprabasal cleft and intraepithelial acantholysis of both the oral mucosa and skin, with granular deposition of IgG and complement C3 in the intercellular area of suprabasal cells. From clinical and histological findings, the patient was diagnosed with JPV. His symptoms started to improve with 3 days of 50-60 mg systemic corticosteroid. To induce clinical remission, intravenous immunoglobulin (IVIG) was administered at a dose of 2 g/kg for three consecutive days, with 20 mg oral prednisolone daily. After 1 week, his skin lesions had improved markedly and the pain of the oral mucosa and skin was also relieved. Intravenous immunoglobulin was recently reported to reduce the side effects of corticosteroids. Although further follow-up of our patient is necessary to determine whether IVIG alone can induce complete clinical remission of JPV, the results suggest that IVIG is an effective adjuvant therapy for JPV.