Pemphigus herpetiformis (PH) is a rare pemphigus variant. The clinical features of this form of pemphigus resembles dermatitis herpetiformis, however, histological examination and immunofluorescence yield findings diagnostic for pemphigus. A 5-year-old female patient with clinical features suggestive of dermatitis herpetiformis is reported. Histopathological exam showed skin with subcorneous blisters, epidermal spongiosis containing predominantly neutrophils, few eosinophils and rare acantholytic cells. Direct immunofluorescence showed intercellular deposits of IgG and C3. The skin lesions responded poorly to dapsone associated with systemic corticosteroid therapy. A complete remission of bullous lesions was obtained with azathioprine and immunosuppressive doses of systemic corticosteroids (prednisone). A case of PH in childhood is reported here, emphasizing the rarity and young age of onset.