Pemphigus is an autoimmune blistering disease that is rare in children and adolescents, but all clinical types of pemphigus occur in children. To our knowledge 105 cases of pemphigus in children have been reported till now. In current review we described the clinical manifestations, sites of involvement, treatment and course of the disease. We would like to emphasize that although juvenile pemphigus occurs rarely it should always be considered in differential diagnosis of bullous diseases in children. Early diagnosis and therapy influence the course of the disease toward better prognosis.