Coarctation of the aorta was first described by Morgagni in 1760, and in its simplest form refers to isolated and discrete stenosis of the proximal thoracic aorta. However, coarctation may also be associated with a longer segment of narrowing, with hypoplasia of the transverse aortic arch, or with stenosis of the lower thoracic or abdominal aorta (Ho SY, Anderson RH, Br Heart J 41:268–74, 1979; Price TP, Whisenhunt AK, Policha A, Ayad MT, Gardiner GA, Abai B, et al. Ann Vasc Surg 28(5):1314 e15–21, 2014; Mullen MJ, Heart. 89(1):3–5, 2003). While more severe cases typically present in the neonatal period, aortic coarctation may be diagnosed at any age, either in isolation or in association with other cardiac defects. Crafoord was the first to perform a successful surgical repair of aortic coarctation in 1944 (Kvitting JP, Olin CL, Ann Thorac Surg 87:342–6, 2009). Since then, various surgical and transcatheter approaches have been developed, which have enabled significantly improved outcomes. In this chapter, we will focus our attention on the etiology, evaluation, and management of coarctation of the thoracic aorta and then discuss the less common presentations of abdominal aortic coarctation and pseudocoarctation.