Developmental coarctation, hypoplasia, and occlusion of the abdominal aorta is a rare disease encompassing many differing etiologies and diverse methods of treatment. Long-segment thoracoabdominal aortic occlusion, an extreme manifestation of this disorder, has not previously been reported in children. Two pediatric patients with this entity, a 5- and 13-year-old with uncontrolled hypertension, underwent extensive arterial reconstructions for this entity and provided the impetus for this report. An ascending thoracic aorta to infrarenal aortic expanded polytetrafluoroethylene bypass was undertaken in the younger child. A distal thoracic aorto-bi-iliac artery expanded polytetrafluoroethylene bypass, with implantation of the left renal artery to one graft limb and a right renal artery bypass originating from the other limb, was performed in the older child. There were no major perioperative complications. Both patients were discharged with easily controlled blood pressures. They have remained normotensive at 13 and 14 months follow-up.