Fungal infections of the central nervous system (CNS) are an important cause of morbidity and mortality in HIV-infected patients. Cryptococcus neoformans is the most important fungal pathogen in AIDS patients throughout the world. Other important but less frequent fungal pathogens include Histoplasma capsulatum, Coccidioides immitis, Aspergillus spp., the zygomyces, Blastomyces dermatitidis, Paracoccidioides brasiliensis, and Candida spp. Histoplasmosis, coccidioidomycosis, paracoccidioidomycosis, and blastomycosis are limited to certain geographic areas, whereas the other pathogens are relatively ubiquitous. In the developed world the incidence of invasive fungal infections has declined with the widespread use of highly active antiretroviral therapy (HAART); however, in the developing world, where the vast majority of HIV-infected individuals are not receiving HAART, these pathogens, especially C. neoformans, continue to cause significant morbidity and mortality. Major advances in the management of cryptococcosis in the past few years have led to significant improvements in mortality, but morbidity remains elevated. The optimal management of other, less common CNS fungal infections in AIDS is less well defined due to the paucity of controlled trials. The advent of the worldwide AIDS pandemic led to a sharp increase in the number of cases of cryptococcosis. Further decreases in cryptococcosis have paralleled the decline in other opportunistic complications observed in the HAART era. In much of the developing world C. neoformans remains an important cause of morbidity and mortality and is one of the most common AIDS-defining conditions.