A case of bilateral orbital mass and xanthelasma of the eyelids is presented. Histology confirmed it to be a form of histiocytosis, possibly an Erdheim-Chester disease. This was further confirmed by the presence of a retroperitoneal mass and hydronephrosis, which resolved with treatment. A review of the literature on and pathological features of this rare fatal disease is presented.

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http://dx.doi.org/10.1076/orbi.18.2.99.2714DOI Listing

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