To evaluate the course, use of diagnostic modalities, management, and outcome of aortic thrombosis associated with umbilical artery catheterization, we reviewed 20 cases of aortic thrombosis diagnosed by ultrasonography (n = 16) or aortography (n = 4) over 4 years. Fourteen of 20 infants had severe perinatal asphyxia, suggesting that asphyxia predisposes to aortic thrombosis. Ultrasonography provided information about the size, location, and configuration of the thrombus and was useful in following thrombus size with therapy. Radionuclide renography-scintigraphy demonstrated abnormal renal function in all 11 patients scanned. Six patients with minor thrombosis (hypertension as the only sign) improved without specific therapy. Nine infants had moderate thrombosis (multiple signs but normal urine output); all survived with a variety of therapies; two were not given anticoagulant or fibrinolytic agents; three improved with heparin alone; and one had surgery without recurrence of the thrombus. Resolution of moderate thrombosis followed streptokinase therapy in two of three infants. All five babies with anuria from major thrombosis died. Hepatic infarction associated with aortic thrombosis was found in three of three autopsies.