Here we present a 47-year-old male diagnosed with a pseudocoarctation of the aorta and a funnel-like subclavian artery aneurysm with a large orifice and severe aortic valve insufficiency. The patient underwent a two-stage repair for both pathologies. After an aortic valve replacement, postcardiotomy pericardial effusion occurred and was medically managed. Six months later, the patient underwent a distal arcus aorta and subclavian artery replacement with a left posterolateral thoracotomy as the second stage. Due to the strict adhesions, pulmonary veins were not cannulated and an extracorporeal bypass between the pulmonary artery and femoral artery was used for distal body perfusion. The coincidence of subclavian aneurysms and a pseudocoarctation of the aorta is rare and a literature review was performed to identify treatment options for this pathology.