Congenital and acquired ascending aortic aneurysms in pediatric and young adults are generally encountered in those with Marfan, Lowes-Dietz, and Ehlers-Danlos syndromes as well as patients with sinus of Valsalva aneurysms who may require an aortic valve-sparing operation. Surgical management of these patients with aortic root and aortic wall pathology is dependent on aortic leaflet competence, annular size, and presence of dissection. This chapter will focus on whether a valve-sparing or valve-replacement strategy should be performed. In cases where aneurysms result in dissections into the transverse arch and descending aorta, a different and more extensive surgical strategy will be required to reestablish normal unobstructed flow into the brachiocephalic arteries and the descending aorta. Management schemes and complications relating thereto include myocardial preservation, cerebral protection, and establishing normal anatomic integrity. We will also discuss the importance of cardioplegic methods, “debranching” techniques, and operative hemostasis.