Sinus of Valsalva aneurysm (SVA) is a rare condition that has important anatomic considerations and is associated with various other congenital heart lesions. Its expression differs significantly in various ethnic groups. The SVA is that part of the aortic root bound by the aortic cusp, the aortic valve annulus, and the sinotubular ridge. A congenital SVA is a thin-walled expansion of the aortic wall that arises from the base of a single aortic sinus. Less often, it manifests as a simple fistulous tract between an aortic sinus and an adjacent structure. It originates from either the right sinus or the noncoronary sinus of Valsalva and communicates into adjacent low-pressure chambers. When it breaches the base of the heart into an adjacent low-pressure chamber, the result is cardiac distention, large left-to-right shunts, and para-aortic insufficiency. Rarely, it may rupture freely into the pericardial space. The incidence of SVA has been reported to be 0.26 % in Western patients, 1.37–1.5 % in patients from India, and 2.0–3.57 % in patients from Japan.