A 26-year-old woman with a congenital heart condition (Ebstein anomaly) received a replacement heart valve. Six months later, a rare fungal infection caused by Fusarium developed on the new valve, leading to lung complications. Through a combination of surgery to remove the infected valve and antifungal medications, particularly voriconazole, the patient recovered successfully. This case demonstrates that even life-threatening fungal heart infections can be treated with proper surgical intervention and targeted medication.