Central nervous system infections by protozoa constitute a problem of increasing importance throughout the world. This is partially due to the globalization of our society, tourists and business people being more frequently exposed to parasitic infection in tropical countries than in moderate climate countries. Knowledge of epidemiology, initial clinical signs and symptoms, diagnostic procedures as well as specific chemotherapeutic therapies and adjunctive therapeutic strategies is of utmost important in all of these infections and infestations of the nervous systems, be it by protozoa. This article discusses the clinical presentation, diagnosis, and treatment for some of the more common infections of the nervous system caused by protozoans: Naegleria fowleri, Acanthamoeba spp, Balamuthia mandrillaris, Toxoplasma gondii, Trypanosoma cruzi, Trypanosoma brucei rhodesiense, Trypanosoma brucei gambiense and Plasmodium falciparum.