Background: Sinus of Valsalva aneurysms (SOVA) are very uncommon, with an incidence ranging from 0.1 to 3.5% of all congenital heart defects. Such aneurysms account for only 0.14% of all open heart surgical procedures. SOVA are usually diagnosed as an incidental finding or after an acute rupture into an adjacent cardiac structure. Case Report: We report a case of SOVA associated with severe aortic valve regurgitation. Conclusion: Before rupture, SOVA may present with conduction-system abnormalities attributable to erosion into the inter-ventricular septum, thromboembolism originating in the aneurysm sac, and myocardial ischemia attributable to coronary compression.