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Cicatricial pemphigoid in the aerodigestive system is rare, but can be life-threatening. We report a case of total laryngeal stenosis. During an exacerbation, the airway of the patient was obliterated with a cicatrix and necessitated an emergency tracheostomy. Six years later when no recurrence of pemphigoid was confirmed, laryngofissure operations were performed with a satisfactory restoration of laryngeal function. Though otolaryngological treatment in the active phases is symptomatic including tracheostomy, surgical procedures should be encouraged once the disease has reached a remission.
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