Idiopathic midline destructive disease(IMDD) is a subgroup of midline granuloma syndrome, which is a clinical entity characterized by a relentless uilcer ation of the upper air way involving the nose, the palate, and the face, with a fatal outcorne. A 32 year-old male had symptoms of nasal obstruct.ion and rhinorrhea for about 4 months prior to visiting the hospital ad was referred to our Department for evaluation of a verely destruct.ive skin lesions on his face. The patient, revealed unique clinopatholcgic featurs compat with IMDD. These were : 1) prescence of locally destructive lesion which were restricted to the upper respiratory tract. 2) abscence of systemic disease durin the follow up peroid, 3) The histologic features consisted of acute and chronic inflarnmation with a variable dearel of necrosis on the examination of repeated biopsy specimens, 4) inabiliy to demonst rate an infectious causative organisms on repeated laboratory tests. (Kor J Dermatol 1992; 30(1): 93-98)