BACKGROUND. Invasive fungal disease (IFD) is a significant cause of morbidity and mortality in immunosuppressed children. We aim to describe the epidemiology and clinical parameters of IFD in a Pediatric Hematology-Oncology Unit (PHOU) with an increasing activity during the last 14 years. METHODS. We retrospectively reviewed the medical records of children (up to 18 years old) admitted for IFD to the PHOU of a tertiary hospital in Madrid (Spain), between 2006-2019. Epidemiological, diagnostic and therapeutic parameters were compared according to the type of infection, study period (considering 3 equivalent time fractions) and outcome. RESULTS. Twenty-eight episodes of IFD occurred in 27 out of 471 children at risk (13 males, median 10 years old). Five episodes of candidemia (all in the first period) and 23 bronchopulmonary mold diseases were registered. Six (21.4%), eight (28.6%) and 14 (50%) episodes met criteria for proven, probable and possible IFD, respectively. Most episodes (71.4%) occurred in high-risk children receiving antifungal prophylaxis. Eight children required intensive care and six died during treatment. Mold infections occurred in older children (126 vs. 21 months; p=0,045), increased over time (p=0.002) and were more common in high-risk compared to low-risk children (87% vs. 0%; p=0.001). There were no differences in mortality rates between periods, types of infection or underlying conditions. CONCLUSIONS. Yeast infections decreased within the study time and mold infections were more frequent in high-risk patients. A rising activity in our PHOU and an increase in the complexity of pathologies were not followed by an increase in mortality rates.