""Idiopathic Midline Destructive Disease(IMDD)"", a specific clinicopathologic subgroup of Midline Granuloma Syndrome(MGS), was first described by Tsokos et al. in 1982 and its clinical feature was characterized by relentlessly progressive localized destruction restricted to the upper respiratory tract without systemic involvement and excellent prognosis with radiation therapy. Histopathologic characteristics of IMDD was defined as an admixture of neutrophils, lymphocytes and plasma cells, with new capillary formation, a variable amount of necrosis and no evidence of a neoplasm or an infectious origin. Recently, the authors experienced a case of IMDD proved histopathologically and clinically.