The case of a 57-year-old man with a congenital aneurysm of the right sinus of Valsalva dissecting into the interventricular septum is presented. The patient had a previous heart block and aortic insufficiency. The diagnosis was made by echocardiography and cardiac catheterization. Open-heart surgery was performed, closing the entrance of the aneurysm and replacing the aortic valve. The patient survived surgery and is in New York Heart Association functional class II 3 years after surgical repair. Other cases of this uncommon congenital heart disease are reviewed.