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Osteoma is a tumor of mature bone showing slow growth of well-differentiated bone. It occasionally produces symptoms of space occupying lesion and symptoms of ethmoid osteoma occur earlier than that of osteoma of the frontal sinus. Osteoma of orbital area is uncommon and it may produce ophthalmologic symptoms and cosmetic problems. Treatment remains controversial:open procedures are typically being used, however, small sized ethmoid osteoma are removed by intranasal procedure. Recently, the authors have experienced a case of giant ethmoid osteoma with orbital extension, which was removed with intranasal endoscopic and external ethmoidectomy approach. Hence, we report this case with a review of literature. (Korean J Otolaryngol 2003;46:77-80)