Interruption of the aortic arch is a rare and usually lethal congenital anomaly that is often associated with multiple cardiac malformations. Most neonates with aortic arch interruption perish once the ductus arteriosus closes after birth. However, sporadic cases have been reported to survive into adulthood uneventfully. Here, we report a 19-year-old male with a 3-month history of exertional dyspnea. A series of cardiovascular studies confirmed the presence of aortic arch interruption in conjunction with sinus venosus atrial septal defect and partial anomalous pulmonary venous connection. To the best of our knowledge, such an association has not been previously reported in adults.