Severe systemic Geotrichum capitatum (G. capitatum) infection is rare, especially in Japan. G. capitatum infection has been reported mainly in immunocompromised patients and the prognosis is poor with a mortality rate of approximately 50-75%. Here, we report a Japanese case of systemic G. capitatum infection in a severe neutropenic patient who was receiving chemotherapy for acute myelogeneous leukemia with multilineage dysplasia. G. capitatum was isolated from blood cultures, and also formed multiple nodular lesions in lung fields. The infection was successfully cured with a combination of amphotericin B, itraconazole, and voriconazole.