We report a case of a 33-year-old man with infective endocarditis of both atrioventricular valves coexisting with a congenital heart defect: atrioventricular canal defect. Transthoracic and transesopageal echocardiography showed complete atrioventricular canal defect and vegetations affecting both the normal mitral and tricuspid valves. The patient received a combined antibiotic therapy and was qualified for cardiosurgical correction.