PURPOSE OF REVIEW: Balamuthia mandrillaris infection of the skin and central nervous system has been increasingly reported in the last decade, making this entity a genuine emerging disease. The ability of the clinician in recognizing the skin lesion early in the course of the disease may lead to a successful therapeutic intervention in an otherwise fatal disease. RECENT FINDINGS: In the past years, advances have been made regarding knowledge about the ubiquity of the ameba in the environment, its worldwide distribution (with higher prevalence in South America), the patients at risk (particularly those of Hispanic origin), the diagnostic methods (including those based on molecular biology) and the different therapeutic strategies that have resulted in survival of patients. A recent report dealing with organ transplant transmission of this infection has made it a subject of interest in transplant medicine. SUMMARY: The present review will allow readers from different fields (clinician, dermatologist, neurologist, infectious disease and transplant specialist) to become familiar with the clinical aspect of the disease, including diagnosis and therapy. © 2011 International Society of Nephrology.