Aortic stenosis at young adult age is usually the result of a stenotic bicuspid aortic valve, which is the most common cardiac congenital anomaly. In clinical practice, exercise and pregnancy are important topics. Furthermore, the timing of intervention is under debate, as little information is available on the natural history and outcome after aortic valve replacement in these young adults. In older patients, there is a trend towards earlier intervention. With the increased knowledge of the pathophysiology of aortic stenosis, studies have focused on the dilatation of the ascending aorta with risk of dissection. Recently, it has been suggested that pharmacologic treatment of aortic stenosis could be beneficial for these young adults.