Dissection and rupture of the aorta account for 20% of death in the natural history of aortic coarctation. We describe here in four patients in whom an ascending aortic aneurysm was associated with aortic coarctation. Three patients had aortic valve incompetence. In two cases there was a dissection. This had not been recognized preoperatively in one, in whom the intimal tear was small; in the other patient, with dissection and shock, the associated mild coarctation was demonstrated only at autopsy. Surgical treatment of patients with aortic coarctation and associated aortic aneurysm must include resection of both the stenotic isthmus and dilated section of the aorta, because of a documented poor prognosis. Furtherly, when evaluating patients with aortic dissection a coexisting coarctation although infrequent must be ruled out. If such an association is identified in emergency cases the dissected aorta should be repaired first, employing a suitable technique (double arterial cannulation, above and below the isthmus) in order to ensure adequate perfusion. When there is no acute dissection, repair of the coarctation should be undertaken first.