Abstract: Objective　To explore the clinical characteristics, diagnosis and prognosis of invasive fungal disease (IFD) after chemotherapy in children with acute leukemia (AL). Methods　The clinical data of 15 AL pediatric patients combined with IFD were retrospectively analyzed. Results　In the 15 pediatric patients, there were 4 cases (26.7%) of confirmed IFD, 8 cases (53.3%) of clinically diagnosed IFD and 3 cases (20.0%) of suspected IFD. The infection sites were lung (8 cases), liver (3 cases including 1 case combined with splenic fungal infection), sinus (2 cases) and fungemia (2 cases). All patients were given antifungal treatment. Four patients achieved complete response and 2 patients achieved partial response. The total effective rate was 40.0% (6/15). One patient was in stable condition and one patient was in progress. The total ineffective rate was 13.3% (2/15). Seven patients died and the total mortality was 46.7% (7/15). Conclusions　The most common IFD in children with AL after chemotherapy is invasive pulmonary fungal infection. The pathological diagnosis of IFD is difficult. IFD is dangerous with high mortality.