This report describes a case involving the rare combination of persistent truncus arteriosus type A2, double aortic arch, and mitral stenosis. At the age of 26 days, the patient underwent division of the right-sided aortic arch with tracheal compression and bilateral pulmonary banding. Fontan completion was successfully achieved after separation of the pulmonary artery from the arterial trunk, atrial septostomy, and modified Blalock-Taussig shunt at the age of 7 months and bilateral bidirectional Glenn anastomosis at the age of 1 year and 3 months. At this writing, the patient is doing well 2 years and 6 months after Fontan completion.