We studied the incidence among, risk factors for, and survival of adult patients with acute leukemia and hepatosplenic candidiasis during the period 1980 to 1993. Of 562 adult patients with acute leukemia, 38 (6.8%) had hepatosplenic candidiasis. The incidence of infection increased fivefold during the study period. The incidence was higher among patients with acute lymphatic leukemia (11.3%) than among those with acute myeloid leukemia (5.1%) (P = .01). The median survival was 9.5 months, and by the end of follow-up, 74% of patients had died. Patients whose leukemia was in remission before the last cytotoxic treatment preceding hepatosplenic candidiasis survived longer than did patients with newly diagnosed or refractory or relapsed leukemia (P = .0065). Eleven patients died within 3 months after the diagnosis of the infection: 7 of 16 with newly diagnosed leukemia, 4 of 10 with refractory or relapsed leukemia, and 0 of 12 with leukemia in remission (P = .028). In all of the patients who died within 3 months, infection was found at autopsy. In conclusion, the incidence of hepatosplenic candidiasis has significantly increased since 1980, and the outcome for patients with this infection is related to the stage of leukemia.