Candida albicans endocarditis occurs mostly in patients with congenital heart disease; open heart surgery is the greatest predisposing factor. We report on a child with truncus arteriosus communis and a large Candida vegetation within the prosthetic pulmonary valve, causing severe right ventricular outflow tract obstruction. Treatment was performed successfully by surgery and administration of liposomal amphotericin B (AmBisome) and 5-flucytosine.