RESUMO
Adult Onset Xanthogranulomatous Disease represents an umbrella term encompassing 4 distinct entities with partially overlapping clinicopathological features: adult-onset xanthogranuloma, adult-onset asthma with periocular xanthogranuloma, Erdheim-Chester disease, and necrobiotic xanthogranuloma. They are characterized by a predilection to affect the orbit and ocular adnexa and special histopathological features, in particular infiltrates comprising non-Langerhans-derived foamy histiocytes and Touton giant cells. The appearance of the eyelid lesions is virtually diagnostic, producing readily recognisable diffuse, yellow plaques. Affected patients should be investigated and reviewed regularly for systemic disease. We would like to present a case of 35-year-old male patient with Adult-onset xanthogranulomatous disease [AOXG] associated with vitiligo. This case is reported for its rarity.