Leishmaniasis is a vectorborne disease caused by Leishmania protozoa, which is a major health problem and a neglected disease common in many regions of the world. Leishmania is an intracellular parasite transmitted by sand flies that causes clinical manifestations ranging from a severe and potentially fatal disease named visceral leishmaniasis to less severe but in many cases disfiguring diseases that mainly affect the skin or mucosal tissues, known as cutaneous leishmaniasis. Despite the detection of Leishmania parasites in the brain and cerebrospinal fluid of human patients and dogs, epidemiological data, as well as information about the mechanisms of central and peripheral nervous system alterations, are poorly described. This review is focused on the current knowledge about the neurological manifestations and immunopathogenic mechanisms in human patients and animals infected with Leishmania.