Epidermolysis bullosa dystrophica is a rare hereditary skin disease of infancy in which minor trauma causes blister formation. We report a rare case of epidermolysis bullosa dystrophica (recessive) with a stenosis of the larynx due to epiglottic deformity. We performed a tracheotomy, and we detained a trachea aperture in the long term this time. In a characteristic of a disease though stimulation to the trachea causes the erosion on a trachea mucous membrane, postoperative course was uneventful and we are going to observe it in future.