Coarctation of the aorta is a congenital aortic stenosis, which is usually diagnosed and sur­gically treated in childhood. Diagnostic work-up for initial diagnosis and follow-up in these patients comprises clinical examination, electrocardiogram, chest X-ray, echocardiography, magnetic resonance imaging or computed tomography of the aorta, exercise testing and 24-hour blood pressure study. These investigations are used to evaluate the morphology of the defect and to determine its hemodynamic significance. The treatment of choice is surgical repair or percutaneous catheter intervention. Despite successful surgical or percutaneous repair, patients with coarctation of the aorta are not cured. Late complications (systemic arterial hypertension, recoarctation or residual coarctation, and dilatation or an aneurysm of the ascending aorta) lead to increased morbidity and mortality. However, regular follow-ups and prompt treatment of late complications improve survival and quality of life in these patients.