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Wegeners granulomatosis is an uncomon idiopathic, multisystem disease characterized by necotizing granuloma and vasculitis affecting small to medium sized vessels. It is classified into a classic form and a limited (localized) form based on the anatomic ecently, it has been made possible for the diagnosis of this disease to be carried out in an earlier stage by measurement of the ANCA (Antineutrophil cytoplasmic antibodies) and the result of treatment has improved since cyclophosphamide therapy. Wegeners granulomatosis in children has been extremely rare. The authors have observed 1 case of limited form of granulomatosis in a 12 year-old female child and report this with a literature review. (Korean J Otolaryngol 2002;45: )