A patient with dilated cardiomyopathy and supraventricular tachycardia presumed to be of sinus origin was referred for cardiac transplantation. The extreme rate of the tachycardia during exercise, profound fluctuations in heart rate, and the presence of an abnormal P wave axis suggested the diagnosis of incessant ectopic atrial tachycardia rather than compensatory sinus tachycardia. Electrophysiologic study with endocardial activation sequence mapping confirmed the diagnosis of an ectopic left atrial automatic tachycardia, after which surgical cryoablation of the left atrial focus was carried out successfully and sinus rhythm was restored. Serial radionuclide angiocardiograms obtained before and after surgery demonstrated a very rapid recovery of left ventricular function to nearly normal within the first month after surgery, followed by further improvement to normal over the next several months. The diagnosis of tachycardia-related cardiomyopathy should be seriously considered in any patient with apparently end-stage dilated cardiomyopathy and persistent resting tachycardia.