Chaotic atrial rhythm in infants has been defined similar to multifocal atrial tachycardia in adults, implying a multifocal etiology. However, its ECG appearance resembles atrial fibrillation, which sometimes has a unifocal ectopic mechanism amenable to catheter ablation. Curative focal radiofrequency ablation was performed in a 4-month-old infant with chaotic atrial rhythm and dilated cardiomyopathy. Left ventricular function subsequently returned to normal. Reversibility of associated cardiomyopathy supports aggressive rhythm management of chaotic atrial rhythm. In this patient, the unifocal origin allows insight into the pathophysiology of the rhythm and demonstrates the potential utility of catheter ablation for refractory cases.