Balamuthia mandrillaris is a free living amoeba that can be isolated from soil. It is an emerging pathogen causing skin lesions as well as CNS involvement with a fatal outcome if untreated. The infection has been described more commonly in inmunocompetent individuals, mostly males, many children, and with a predilection for population with Hispanic background in cases occurring in the United States. Except for Africa, all continents have reported the disease, although a majority of cases are seen in North and South America. In published reported cases from North America, most patients will debut with neurological symptoms, where as in countries like Peru, a skin lesion will precede other symptoms. The classical skin lesion is a plaque, mostly located on face or knee. Diagnosis requires a high level of suspicion. Therapeutic strategies require a multidrug approach, than includes at least one amebicidal drug, and prolonged periods of treatment. © Springer Science+Business Media, LLC 2012.