During the past ten years, we have surgically managed seven neonates who developed total occlusion of the distal aorta due to umbilical artery catheters. All patients experienced symptoms of congestive heart failure. Five patients presented with severe hypertension, and all of these had aortorenal involvement: three infants had aortorenal thrombosis and two infants had infrarenal aortoiliac thrombosis with suprarenal extension of thrombus. Two infants had aortoiliac thrombosis with clot confined to the infrarenal aorta. Aortic thrombosis imposes an additional severe hemodynamic insult in these already seriously ill infants. Survival in this group of patients depends upon prompt recognition of this problem, effective surgical correction, and careful perioperative management. Our experience suggests that this diagnosis should be entertained in the infant presenting suddenly with congestive heart failure, hypertension, or lower limb ischemia after umbilical artery catheterization. The diagnosis is preferably confirmed by real-time ultrasound and/or radionuclide flow scan, although aortography may sometimes be necessary. Surgical management includes early transabdominal aortotomy with thrombectomy. Prompt thrombectomy resulted in the survival of six patients. One infant died in acute renal failure. Renal function and leg perfusion is satisfactory in the remaining patients, although one child required later operative correction of renovascular hypertension. Two additional patients needed prolonged postoperative antihypertensive therapy for 14 to 34 months before this problem resolved. Long-term follow-up is necessary for managing renovascular hypertension and monitoring lower extremity perfusion.