Summary: Introduction: There has been an increased number of cases of fungal diseases of the CNS during the past few years. This paper review current literature about these conditions, with emphasis on recent advances on diagnosis and therapy. Development: While some fungi may cause disease in normal hosts, most of these microorganisms are opportunistics and affect immunocompromissed hosts. With the exception of Candida albicans, that is a normal inhabitant of the intestinal tract, most fungi enter the body by inhalation or through skin abrassions. Common fungal diseases include aspergillosis, blastomycosis, candidiasis, coccidioidomycosis, cryptococcosis, histoplasmosis, mucormycosis, paracoccidiodomycosis, and phaeohyfomycosis. In general terms, fungal invasion of the CNS may produce one or more of the following clinical syndromes: subacute or chronic meningitis, encephalitis, parenchymal brain abscesses or granulomas, stroke, or myelopathy. Diagnosis may be difficult on clinical grounds, and data provided by neuroimaging studies or CSF examination is non-specific. Definitive diagnosis usually rest on the demonstration of the causal agent on body fluids or tissue samples. Early administration of amphotericin B, associated or not with the new azoles, is indicated to arrest the often fatal course of these conditions. Conclusion: Formerly considered rare diseses, the AIDS epidemic and the widespread use of corticosteroids and citotoxic agents, have caused an increase in the prevalence of CNS mycosis. Development of potent antimycotic drugs have improved the prognosis of fungal diseases of the CNS. However, due to diagnostic delays or the presence of debilitating conditions, some patients still have torpid clinical courses despite proper therapy