Although laryngeal stenosis caused by cicatricial pemphigoid (CP) is a well documented entity, reports about its surgical treatment are scant. This may be due to the reluctance of the surgeons to excise the scarring, knowing the recurrent nature of the disease. In this paper we report a case of severe laryngeal stenosis caused by CP which necessitated a tracheostomy. When the disease had stabilized, laryngeal airway was restored following open surgical excision and stenting. The historic, clinical, histologic, immunopathologic and therapeutic features of CP are presented with special reference to the laryngeal lesions.