A 6-year-old child presented with left sinus of Valsalva aneurysm opening in right atrium. Origin of sinus of Valsalva from left aortic sinus and its opening into right atrium is extremely rare. The anomaly was corrected surgically by patch closure at the aortic end. Follow-up echocardiography did not reveal any residual shunt in right atrium. (Indian Heart J 2005; 57: 73–75)

Key Words: Congenital heart disease, Sinus of Valsalva aneurysm, Aortic-cardiac fistula