Trichosporon beigelii fungemia with localized visceral involvement or dissemination is a life-threatening infection in patients with granulocytopenia. We report three cases of T. beigelii fungemia in patients with acute leukemia seen at the National Taiwan University Hospital. Two patients were in the granulocytopenic state after cytoreductive chemotherapy, while one was in complete remission. Fever persisted or recurred despite conventional antibiotic treatment when fungemia developed. Cutaneous trichosporonosis was noted in one patient, which was misdiagnosed as candidiasis. An indwelling catheter constituted the likely portal of entry in two patients. Therapeutic response to amphotericin B is generally not achieved in patients without recovery of neutrophil counts and in those with retained catheters.