A case of bullous pemphigoid (BP) in a five-year-old girl is reported. IgG deposits at the epidermal-dermal junction and high titres of serum IgG (1/10000) reacting with the epidermal-dermal junction of rabbit esophagus were the two criteria that led to the diagnosis of BP. Clinically and histologically this case had at first been diagnosed as dermatitis herpetiformis or benign chronic bullous disease of childhood. This emphasizes the importance of immunological criteria for classifying chronic bullous diseases in children. Our patient did not respond to sulfones and prednisone was given for thirteen months. No relapse occurred during the first year following prednisone withdrawal. Our observation is the twenty-first case of immunologically proven BP in childhood. The twenty-one cases are reviewed in order to analyze the features of the disease in children.