A sinus of Valsalva aneurysm is an uncommon congenital defect, which requires appropriate diagnosis with either echocardiography, magnetic resonance imaging or contrast angiography. Treatment consists of aortic valve repair. We describe a young woman with an aneurysm of the non-coronary sinus of Valsalva, an atrial septal defect and pulmonary insufficiency. The different imaging techniques and possibilities of surgical correction are described.