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Epidermal necrolysis (EN) is a rare, but potentially life threatening disease, characterized by epidermal necrosis and sub-epidermal detatchment, and is predominantly medication-induced. Methazolamide is a sulfonamide derivative and carbonic anhydrase inhibitor used for lowering of intraocular pressure in glucomatous patients. Common side effects of methazolamide include metabolic acidosis, hypokalemia, tinnitus, transient myopia, and renal calculi; however, EN caused by methazolamide is very rare. We report on two cases of EN induced by methazolamide treatment and review previously published cases. (Korean J Dermatol 2011;49(6):533∼537)