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Hereditary angioneurotic edema (HAE) is an autosomal dominant disease that results from the deficiency of C1 esterase inhibitor (C1-INH) function. Urgent proper intervention of the upper airway is warranted to save the life of patients with the disease, which often causes acute airway obstruction. We present, with a review of literature, a case of 37-year old woman with HAE of larynx. (Korean J Otolaryngol 2006;49:464-7)