Abstract Background Subdiaphragmatic aortic diseases in children are rare and form a heterogeneous group. The pediatric patient presents unique challenges because of their size, concerns about proper timing and conduit for repair, and anticipating expected growth. Methods We performed a retrospective review of operations involving the abdominal aorta and called branches in children between January 2003 and April 2007, focusing on the details of preoperative evaluation, operative technique, and outcomes. The pertinent literature is reviewed. Results Twenty-two children (age, 2 days to 17 years) were included. Mean follow-up was 28 months. Aneurysms were seen in 5 children; the remainder had stenotic disease. Aneurysms were typically asymptomatic and diagnosed incidentally, whereas stenotic lesions most commonly presented with hypertension (HTN). Fourteen complex vascular repairs were performed. All of the children with aneurysms underwent prompt surgery. The children with stenoses had operations for poorly controlled HTN, claudication, and/or mesenteric ischemia. Most patients with stenotic disease were treated medically for HTN and were followed closely while awaiting optimal size and availability of autogenous conduit for reconstruction. Cryopreserved allograft was used in 3 of the aneurysm operations. Dacron grafts were used to repair 5 aortic stenotic lesions. Renal and mesenteric revascularizations were performed with saphenous vein grafts. Pediatric, general, and transplant surgeons and nephrologic and cardiologic teams were integral to evaluation and management. No major operative complications occurred. Conclusion Proper management of pediatric aortic vascular disease requires a multidisciplinary approach. It is best to use autologous grafts whenever possible. Children with stenotic disease should be treated medically for hypertension until they are large enough for an autologous graft reconstruction. Children with aneurysmal disease are at risk for embolism and thrombosis and therefore usually treated immediately using artificial graft material, if necessary.