The combination of coarctation of the aorta in the presence of severe aortic insufficiency poses a serious clinical problem. Although successful single- and two-stage repair for combined coarctation in the presence of severe aortic regurgitation has been described, the surgical management of this lesion remains particularly difficult. The analysis of larger series of patients operated upon for coarctation reveals significant early mortality rate in patients with associated severe aortic insufficiency. Although the exact cause of the acute left ventricular failure remains unclear and is a matter of debate, one can assume that changes in the haemodynamics, resulting in global myocardial ischaemia from impaired coronary blood supply or a massive volume overload of the left ventricle after the correction of the coarctation, could have led to myocardial irritability and left ventricular failure. We present a three-stage repair with subtotal relief of the coarctation by balloon angioplasty and stenting first; elective aortic valve replacement in a second stage and finally total balloon dilatation of the residual stenosis at the previously subtotal dilated coarcted segment.