Aortic coarctation is a congenital heart disease that usually presents and is treated in the childhood. The aortic coarctation is often associated with concomitant cardiac pathologies, such as aortic stenosis and bicuspid aortic valve. We report the case of a 56-year-old man, admitted in our cardiologic unit, for sudden onset of chest pain, dyspnea and syncope. Aortic coarctation with aortic valve stenosis was diagnosed. The aortic valve was successfully replaced in the first stage; the coarctation had to be treated in a second time.