Aorto-cameral fistula, either congenital or acquired, is an abnormal connection between the ascending aortic root and one of the cardiac chambers. We report a case of a 61-year-old male with history of atrial fibrillation and 2 radiofrequency ablation procedures, referred to us for surgical Cox Maze procedure. Preoperative cardiac computerized tomography revealed a fistulous connection between the aortic root and the right atrium. Later, the patient underwent successful surgical closure of the fistula during the Cox Maze procedure. In this paper, we also discuss the clinical presentation, natural history, anatomy of the interatrial septum, and management of aorto-cameral fistula.