Aortic coarctation is rarely associated with an aneurysm of the ascending aorta and an aortic coronary fistula. In this study, we report the case of a 52-year-old man undergoing surgery for an isthmic coarctation who also had an aneurysm of the initial portion of the aorta and an aortic coronary fistula. The diagnosis was clinically suspected and confirmed by vascular catheterism. The first operative stage consisted of treating the coarctation. The second stage was performed two months later to remove the aneurysm and replace the ascending aorta and the aortic valve with a prosthesis. The coronary arteries were then reimplanted. The postoperative results were quite favourable.