Candida prosthetic endocarditis (CPE) is an uncommon and fatal complication in adults with congenital heart disease. The current guidelines for the management of fungal endocarditis recommend a combination of surgical and medical therapy. However, it still remains uncertain when surgical management in CPE patients should be performed. Therefore, the prognosis of CPE patients is very poor. Here we report a case of CPE in a 31-year-old woman who had undergone surgical repair for tetralogy of Fallot during childhood and pulmonary valve replacement at the age of 21 years. She underwent re-pulmonary valve replacement after being sufficiently sterilized with a 5-week course of antifungal medical therapy, leading to clinical improvement. In CPE patients, it is necessary to perform surgical therapy while suppressing the activity of fungi as much as possible.