Fungal endocarditis (FE) is a rare but fatal disease. The incidence of infective endocarditis (IE) in hemodialysis (HD) patients is thought to be obviously higher than that in the general population. Moreover, IE occurs more likely in HD patients with catheters. We reported a case of IE caused by Aspergillus flavus (AF) in a 36-year-old woman on HD. The patient was in good condition after valve replacement surgery and 9 months of voriconazole therapy. The diagnosis and treatment strategies of FE in HD patients were discussed.