Sinus of Valsalva aneurysms are rare cardiac anomalies which may be acquired or congenital. The congenital aneurysm is more common than the acquired form, with an incidence ranging from 0.1 to 3.5% of all congenital heart defects. Acquired aneurysms may result from trauma, endocarditis, syphilis, Marfan's syndrome, and senile-type dilatation in which the three sinuses dilate as a result of the normal aging process. This review focuses on both congenital and acquired aneurysms with particular attention to the noninvasive diagnosis of this anomaly.