Pseudoaneurysm of the ascending aorta is rare but life-threatening. We report a case of a primary pseudoaneurysm originating from the ascending aorta that recurred six months after surgical treatment, and the patient eventually died of shock due to a rupture of the pseudoaneurysm. Furthermore, to the best of our knowledge this is the first reported case of Relapse of ascending aorta pseudoaneurysm to pulmonary arteriovenous fistula after first resection.In this article, we aim to provide detailed information on the different etiologies and presentations of pseudoaneurysms of the ascending aorta, highlighting the possibility and severity of pseudoaneurysms occurring in young patients.