Ebstein's anomaly is defined as an apical displacement of the attachment of the septal tricuspid valve leaflet from the right atrioventricular annulus that exceeded 1.2 cm in length. Patients with Ebstein's anomaly are known to have a high potential for developing arrhythmia, in the vast majority, of the tachycardia type. Infective endocarditis is characterized by ulcerovegetational lesions that result from the graft of a microorganism, usually bacterial, on the valvuler endocardium (native valve endocarditis) or on a prosthesis (prosthetic valve endocarditis). Ebstein's anomaly with tricuspid regurgitation is also thought to be a predisposing condition for infective endocarditis. In this case, we report a patient who presented with atrial flutter and infective endocarditis due to residual pacemaker lead and Ebstein's anomaly.