Lodderomyces elongisporus has been recently identified in the literature as an infrequent human bloodstream isolate, commonly mistaken for a non-albicans Candida. A case of Lodderomyces endocarditis in an intravenous drug user is described. To our knowledge, this report highlights the first documented case of Lodderomyces as a cause of endocarditis and summarizes the susceptibility patterns in the reported literature. All isolates reported so far have fluconazole MICs of ≤0.25 µg ml(-1).