The term ""Steven-Johnson syndrome"" is commonly used to describe an illness in which the cutaneous lesion of erythema multiforme are accompanied by bulla, ulceration of the conjunctiva, mouth and genitalia with systemic upset and fever. Other names that have been given to the same condition include ectodermosis erosiva pluriorificialis, mucocutaneous ocular syndrome, erythema multiforme exudativum majora and dermatostomatitis. The clinical manifestations of Steven-Johnson syndrome were rarely observed in the otolaryngologic fields. The author have recently experienced one case of the Steven-Johnson syndrome and report here now with the literatures.