Truncus arteriosus is a rare form of congenital heart defect. Among cases, 10% to 20% are associated with arch anomalies, such as interruption, coarctation, or patent ductus arteriosus. We treated a rare case of combined truncus with complex arch anomaly. The patient was a 2-month-old girl weighing 3.8 kg. Echocardiogram and computed tomogram showed interrupted aortic arch, truncus arteriosus, persistent fifth aortic arch, and its coarctation. One-stage total correction was performed under cardiopulmonary bypass without circulatory arrest. The operation consisted of a Rastelli procedure with a 12-mm valved conduit, coarctoplasty, and intraventricular baffling. The patient recovered well without complications.