A 64-year old man was admitted to our hospital with a diagnosis of aortic stenosis. Pre-operative chest CT revealed pseudocoarctation of the aorta with a hypoplastic aortic arch, elongation and kinking of the aortic arch and proximal descending aorta. There was also a large aneurysm from the distal arch to descending aorta. We performed a single-stage repair of the aortic lesion from the ascending to the descending aorta with aortic valve replacement. For the surgical approach, transverse clamshell incision was applied safely. Concomitant aortic valve replacement in surgical repair of pseudocoarctation and thoracic aneurysm was rare, and clamshell incision seemed beneficial in such single-stage repair from the aortic root to the descending aorta.
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