Abstract
A three-month-old female infant with a structurally normal heart was diagnosed with fungal endocarditis of the mitral valve with cerebral embolism. After antifungal therapy and a valve-sparing operation with complete removal of the fungal vegetations, a relapse with complete destruction of the valve leaflets and severe mitral regurgitation with decompensated heart failure occurred three months later. A second operation with successful mitral valve replacement was performed. Non-compliance with the anticoagulant treatment with vitamin K antagonist led to thrombosis of the mitral valve prosthesis one year later, and the child died from acute pulmonary edema.