Patients with the Stevens-Johnson syndrome or erythema multiforme exudativum may present to the otolaryngologist because of involvement of the mucosa of the upper aerodigestive tract. Stevens-Johnson syndrome is a symptom complex of uncertain cause that affects the eyes, skin, and mucous membranes throughout the body. The epithelial lesions progress from macules to bullae that desquamate, form pseudomembranes, and finally heal. Generally there is a prodromal period with fever and symptoms of an upper respiratory tract infection. The literature related to the otolaryngologic manifestations of the disease is reviewed and a case of Stevens-Johnson syndrome with early involvement of the supraglottic larynx is presented. The otolaryngologist should be aware of this syndrome and should be prepared to manage the possible airway compromise.