Background: Atrial fibrillation (AF) is a common cause of tachycardia-induced cardiomyopathy (TIC). This study evaluated the outcomes of the Cox-Maze IV procedure in patients with TIC and significant left ventricular dysfunction. Methods: Between January 2002 and January 2017, 37 consecutive patients with a left ventricular ejection fraction (LVEF) of 0.40 or less underwent stand-alone surgical ablation of AF. After dilated and ischemic cardiomyopathies were excluded, 34 of 37 patients met the criteria for the diagnosis of TIC. Results: Patients were a mean age of 56 ± 11 years, and 24 (70%) had long-standing persistent AF. The median AF duration was 72 months (interquartile range, 9 to 276 months). Seventeen patients (50%) had at least one catheter-based ablation that failed. Mean LVEF was 0.32 ± 0.08. There were 11 patients (32%) with New York Heart Association Functional Classification III/IV symptoms. There was one (3%) 30-day mortality caused by a pulmonary embolus, despite full anticoagulation. At 12 months, freedom from atrial tachyarrhythmias on or off antiarrhythmic drugs was 94% and 89%, respectively. Postoperative echocardiograms were available for 27 of 33 patients (82%). The LVEF improved to a mean of 0.55 ± 0.08 (95% confidence interval, 0.51 to 0.58; p < 0.001). Of the 11 patients with New York Heart Association Functional Classification III/IV symptoms, 8 patients were in class I/II at the last follow-up (p = 0.02). Conclusions: Restoration of sinus rhythm with the Cox-Maze IV was associated with significant improvement in the LVEF in patients with AF and TIC. This retrospective study illustrates the efficacy of the Cox-Maze IV in this patient population both at restoring sinus rhythm and improving ventricular function. Patients with TIC and poor left ventricular function in whom other treatments have failed should be strongly considered for surgical ablation.