Epidermolysis bullosa acquisita (EBA) is rarely reported in childhood, but we now describe a 6-year-old Korean girl with the condition. She presented with multiple tense bullae annularly distributed on the perioral, periorbital and genital areas, and was successfully treated with dapsone. The clinical and histological features were similar to those of chronic bullous dermatosis of childhood. We review seven previously reported childhood EBA cases and contrast their features with those of adult EBA. We suggest that some childhood EBA is different from the adult form and shares features with chronic bullous dermatosis of childhood.