The adult orbital xanthogranulomatous disease (AOXD) is a rare group of orbital and ocular heterogeneous disorders. It is characterized by the proliferation of non-Langerhans (type II) histiocytes. 1,2 According to the severity of systemic involvement, it is classified into four subgroups as adult-onset xanthogranuloma (AOX), adultonset asthma with periocular xanthogranuloma, Erdheim-Chester disease, and necrobiotic xanthogranuloma (Table 1). While the most common subtype is reported as necrobiotic xanthogranuloma, the least common type is reported as AOX, as seen in our case. 1,2 Although there are some histological differences and distinctive systemic associations in these four subgroups, each of them is characterized by the infiltration of foamy histiocytes and giant cells of the Touton type, which are usually negative for S100 protein and CD1a, and positive for CD68. 2