An unusual systolo-diastolic heart murmur was discovered fortuitously in a 39-year-old man undergoing a routine check-up. Transesophageal echocardiography gave the diagnosis of Valsalva sinus aneurysm ruptured into the right atrium. Cardiac surgery was successful. We reviewed the literature on this unusual condition, focusing on the pathophysiological, clinical, diagnostic and therapeutic aspects. Transesophageal echocardiography provides the diagnosis.