Congenital aortic atresia is a malformation accounting for 4 - 6% of all congenital heart diseases in children. Left ventricular outflow obstruction due to atresia is common at the aortic valve but rarely has atresia been identified in the descending aorta. We report the case of a 25-year woman who was evaluated for headache and uncontrolled hypertension. CTscan chest showed a short atretic segment in the descending aorta at the isthmus, distal to the takeoff of the subclavian artery. She underwent surgery; a 22 mm Dacron graft was taken and jump graft was placed between the arch of the aorta and the descending aorta, using partial occlusion clamps. Patient tolerated the procedure well and was discharged on ACE Inhibitors and beta blockers, which were then weaned off over a period of one year. She remained stable and had no further complaints.