Aortic stenosis is one of the three most common congenital heart defects in dogs and less frequent in cats. Most dogs or cats have subvalvular type of stenosis; valvular or supravalvular types are less frequent. Heart failure is seldom a consequence of aortic stenosis; most dogs with heart failure have a concurrent disease. The most common accompanying diagnosis is pulmonic stenosis, especially in the Boxer breed. Screening programs seem to have efficiently lowered the incidence of aortic stenosis in dogs. Genetic evidence for aortic stenosis has been shown in Golden Retriever, Newfoundland and Dogue de Bordeaux; however, the genetic background of aortic stenosis at molecular level remains unclear.