Abstract
This report describes the case of a patient who had symmetrical exophthalmos, periorbital xanthelasmas and reduced vision. Next to Wegener's granulomatosis the differential diagnosis of Erdheim-Chester disease is discussed. It concerns a rare systemic histiocytosis of unknown etiology. Above all, the skeleton system with symmetrical long bone osteosclerosis is affected. Manifestations in the area of the orbit have seldom been reported with bilateral retrobulbar infiltrations, exophthalmos, diplopia, compression of the optic nerve and periorbital xanthelasmas.
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