Abstract: Objective　To improve clinically the recognition of fungal infection associated with hemophagocytic lymphohistiocytosis (HLH) in children. Methods　Clinical data of 3 children with fungal infection complicated with HLH in our hospital was retrospectively analyzed. Results　All the 3 cases complained of recurrent fever, 2 cases with cough and one case with vomiting. Hepatosplenomagaly and lymphadenectasis were found in the medical examination. The time of diagnosis of fungal infection through etiological examination was 5 days after admission. It was further diagnosed as hemophagocytic lymphohistiocytosis after failure of effective antifungal therapy. Routine blood test showed the counts of leukocytes were increased in early stage, while the number of platelets and hemoglobin decreased in different degrees. The recovery is not satisfactory using antifungal therapy alone, and 2 of them are gradually aggravated and treated with mechanical ventilation. On the basis of antifungal therapy, 2 cases were treated under HLH-2004 regimen, 1 received dexamethasone treatment. All the 3 cases received intravenous immune globulin, and showed improvement. Conclusions　Fungal infection complicated with HLH in childhood is rare. The effect of simple antifungal therapy on the progression is limited. However, increasing immunosuppressive therapy based on effective antifungal therapy can improve the prognosis.