A 38-year old woman with a dural based lesion.

[erdheim-chester disease]

Isolated intracranial xanthogranulomas arising from the dura mater are extremely rare .We present a case of a symptomatic large right frontoparietal dura based intracranial xanthogranuloma in a 38 -year -old female . Xanthogranulomas are benign non-Langerhans cell histiocytic lesions . They are frequently described in the skin of infants and children . Extracutaneous manifestations especially in the central nervous system are highly uncommon . Dural xanthogranulomas usually arise in association with familial hypercholesterolemia , with Erdheim Chester disease (ECD ), and with Weber-Christian disease . Our case however , had no such associations . In this report , the authors describe the clinical , radiological and microscopic presentation of this case and the differential diagnoses of intracranial xanthogranuloma .