ABSTRACT
PURPOSE: Primary localized orbital amyloidosis is a very rare disease and it may deposit in the local area without any other previous disease. It may also form nodules in the cornea, conjunctiva, and eyelid. It may involve levator aponeurosis and extraocular muscles, and develops the various symptoms such as ptosis, opthalmoplegia and proptosis. We experienced this rare disease and report the case with a review of the literature. METHODS: A 54-year old woman who presented with ptosis, exophthalmos and conjunctival mass was diagnosed to have localized orbital amyloidosis by orbital CT and pathological review of conjunctival mass and orbital lesion. No systemic involvement was noted by systemic work up and we confirmed as primary localized orbital amyloidosis. RESULTS: It is a first case of primary localized orbital amyloidosis in Korea and should be differentiated with other diseases with similar symptoms as above.