The first described case of meningitis.

We describe the first documented case of meningitis caused by Identification of was made on the basis of an arachnoid biopsy with pathology samples sent for fungal internal transcribed spacer sequencing after multiple central nervous system (CNS) fungal culture specimens were negative. After final diagnosis, treatment was transitioned from amphotericin to fluconazole, which, combined with insertion of lumbar drain followed by a permanent ventriculopleural shunt, resulted in significant clinical improvement. Our report reviews the literature of (1) cases of , which almost exclusively describe fungemia or endocarditis; (2) CNS infections caused by , an organism with which was previously conflated; and (3) management of fungal meningitis-associated hydrocephalus.