Three cases of hepatic and/or splenic mycosis in children with acute leukemia are reported. Patients presented with fever not responding to broad spectrum antibiotics during or after prolonged and profound neutropenia. Noteworthy, in 1 case no abnormality in liver function tests was detectable at diagnosis. CT scan showed focal hepatic lesions in all patients. In 1 patient ultrasounds failed to detect hepatic lesions and showed only splenic lesions, while CT scan detected hepatic and not splenic lesions. Definitive diagnosis was performed by open liver biopsy and histological demonstration of fungal invasion. Cultures were negative in all cases. All patients survived with prolonged amphotericin B treatment (from 36 to 40 mg/kg), combined with flucytosine in two cases. In conclusion, this clinical entity should be taken into account by physicians taking care of neutropenic patients, even in the absence of abnormalities in liver function. CT seems to be the method of choice for diagnosis, although ultrasounds might be useful for detecting concomitant splenic lesions.