A boy with epidermolysis bullosa developed progressive laryngeal obstruction after age six months, with tracheotomy necessary at age three years. By eight years, the larynx was completely occluded by web and scar. Stenosis recurred repeatedly over the next seven years, three to six months after each corrective procedure, but laryngeal patency has been better-maintained since age 15. Laryngeal stenosis, presumably secondary to epithelial detachment, although a rare complication of epidermolysis bullosa, can be serious and life-threatening.