Paracoccidioidomycosis (PCM) is an infectious disease, endemic to subtropical areas of Central and South America, caused by the dimorphic fungus Paracoccidioides brasiliensis. It is a chronic disease, mostly affecting adult males, with a mean patient age of 44 years. Central nervous system involvement (CNS PCM) has been found in 13% of the patients with systemic disease. We reviewed the clinical presentation, diagnosis techniques and treatments for CNS PCM.