Aortic thrombosis is a rare and severe condition in neonates which often is revealed by ischemia of lower limbs. We report a case of major aortic thrombosis revealed by renal failure, hematuria and dehydration in a 10-day-old girl. Clinical features consisted in hypertension at upper limbs without ischemic signs of the lower limbs. Diagnosis was made using renal Doppler ultrasound. Thrombosis resolved after thrombolytic treatment and anticoagulation using heparin. The case report is followed by a review of the literature dealing with clinical, etiological and therapeutic aspects of neonatal arterial thrombosis.