A 57-year-old female with acute promyelocytic leukemia was admitted to our hospital. The PML-RAR alpha fusion transcript was reverse transcription polymerase chain reaction. Complete remission was achieved with intensive induction chemotherapy. Then a high fever unresponsive to antibiotics with increased C-reactive protein continued. Abdominal computed tomography revealed multiple low-density lesions in the spleen. Splenic candidiasis was suspected and peroral treatment with itraconazole (200 mg/day) was begun. After the fungal infection was confirmed to be inactive, splenectomy was performed. The splenic tissue showed multiple white or yellow nodules and methenamine silver stain revealed fungal hyphae characteristic to Candida. There was no evidence of relapse of Candida infection. She has been in complete remission for these two years and free of fungal infection. It is indicated from our case that splenic candidiasis in patients with acute myeloid leukemia can be successfully treated with oral administration of itraconazole and subsequent splenectomy, when it is confined in the spleen.