Aneurysms of the sinus of Valsalva are rarely diagnosed cardiac anomalies, occurring in 0.14%-0.96% of patients who have undergone open heart surgical procedures. The most common congenital anomalies accompanying sinus of Valsalva aneurysm (SVA) are ventricular septal defect, bicuspid aortic valve, atrial septal defect, and coarctation of aorta. We report a patient with an unruptured right SVA presenting with severe right ventricular outflow tract (RVOT) obstruction, and coexisting patent foramen ovale (PFO) with a right to left shunt. It could be assumed that the increase in right atrial pressure due to RVOT obstruction had led to a right to left shunt across the patent foramen ovale.