Bullous systemic lupus erythematosus (BSLE) is a rare autoimmune subepidermal blistering disease, with few cases described in childhood. We report a case of bullous systemic lupus erythematosus refractory to corticosteroid therapy in a 16-year-old female who was successfully treated with low dose dapsone. We highlight the rarity and the relevance of skin biopsy for a correct diagnosis of BSLE.