The pathogenicity of the fungus Geotrichum candidum in humans has not been clearly defined. A patient with acute leukemia who developed a fatal disseminated G. candidum infection while neutropenic is described. At autopsy examination, this patient was misdiagnosed on the basis of histopathology as having disseminated candidiasis until G. candidum was isolated from postmortem culture specimens. The emergence of this organism as an occasional pathogen in leukemic patients is of interest and illustrates the importance of obtaining fungal cultures. There is a need for more effective drugs for antifungal prophylaxis and therapy.