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Abstract We present a case of a 36-year-old male patient with known arthrogryposis multiplex congenita and an associated unicuspid aortic valve. The patient later developed a significant aneurysm of the ascending aorta, however refused surgical intervention and missed follow-up appointments for 5 years. During an urgent, general practitioner-initiated transthoracic echocardiography follow-up, a chronic type A aortic dissection was diagnosed as a result of progressive aortic dilatation. Due to the stationary pressure gradients and non-progressive leaflet fibrosis, a conservative approach for to the unicuspid aortic valve was chosen, combined with replacement of the ascending aorta and partial replacement of the aortic arch.