Aneurysms of the sinus of Valsalva (ASVs) are rare. They can be congenital or acquired through infection, trauma, or degenerative diseases. They frequently co-occur with ventricular septal defects, aortic valve dysfunction, or other cardiac abnormalities. Although unruptured ASVs are usually asymptomatic, ruptured ASVs often cause symptoms similar to those of heart failure and produce a continuous, mechanical-sounding murmur. Transsternal or transesophageal echocardiography is usually effective in detecting ASVs. Because symptomatic ASVs pose significant risks for the patient, and because the repair of asymptomatic ASVs generally produces excellent outcomes, surgery is indicated in most cases. The primary goals of surgical repair are to close the ASV securely, remove or obliterate the aneurysmal sac, and repair any associated defects. Operative mortality is generally low except in patients with concomitant bacterial endocarditis or other infections. Late events are uncommon and tend to be related to aortic valve prothesis or Marfan syndrome.