Neurosarcoidosis without systemic involvement is rare and difficult to
diagnose. The case of a 27-year-old man with a 6-week history of head
ache, mental status changes, and polyradiculopathy attributable to hyp
oglycorrheic lymphocytic meningitis is presented. Extensive testing fo
r occult systemic sarcoidosis was negative. The presence of noncaseati
ng granulomatous inflammation was established by open brain biopsy, an
d the patient improved clinically with oral steroid therapy. In indivi
duals with undiagnosed chronic meningitis, brain biopsy may be necessa
ry to rule out isolated neurosarcoidosis.