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Idiopathic granulomatous inflammation is regarded as a histopathologic variant of an orbital pseudotumor and has a similar clinical presentation and treatment. However, the differential diagnosis including sarcoidosis, Wegener's granulomatosis, trauma, and infection must be ruled out. We present a 77-year-old man with a biopsy-proven lacrimal gland granulomatous inflammation of an unknown origin. Management by surgical debulking via an anterior orbitotomy achieved a good result with minimal complications.
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