A 34 year old woman, who had suffered from hypertension for 16 years, presented with systolic blood pressure up to 220 mm Hg and diastolic blood pressure up to 120 mm Hg, accompanied by headaches, dizziness, and paraesthesia in both arms. Auscultation revealed an aortic systolic murmur with radiation to the back. Doppler echocardiography showed a pressure gradient of 66 mm Hg in the descending part of the aorta. The suspicion of congenital postductal aortic coarctation was confirmed by transoesophageal echocardiography. Because of the impression of a complex structure of the stenosis, magnetic resonance imaging and dynamic three dimensional computed tomographic angiography were performed preoperatively where a siphon-like 75 % stenosis with pre- and poststenotic dilation of the aorta were clearly shown. Because of the complex structure of the stenosis, surgery instead of balloon dilatation was recommended. Intraoperatively, the minimal lumen diameter in the stenotic region was 3 mm. A resection of the aorta was performed with an interposition of a Vascutek prosthesis. The patient recovered uneventfully and has no residual hypertension.