A case of a xanthogranuloma arising in the anterior skull base involving th
e bone of the left orbital roof, the dura mater, and the periorbit is prese
nted. The lesion was completely removed by a supraorbital "eyebrow" skin in
cision using frameless stereotactic image guidance. A reconstruction of the
orbital roof was performed using a titanium mesh graft. The neuropathologi
cal investigation revealed all the characteristics of a xanthogranuloma. In
tracranially, xanthogranulomas appear as rare tumors of the dura or choroid
plexus, usually arising in association with histocytosis X or familial hyp
erlipoproteinemia. One case not associated with these diseases is discussed
.