We report a case of congenital midaortic syndrome with bilateral renal artery atresia in a premature female neonate born by way of caesarean section secondary to acute onset of decreased fetal movement and polyhydramnios. The infant required cardiopulmonary resuscitation at birth, and initial echocardiogram exhibited normal intracardiac anatomy and mildly depressed left-ventricular systolic function. Within 24 h, the neonate developed severe systemic arterial hypertension and acute renal failure. Ultrasound demonstrated hyperechoic kidneys and a hypoplastic abdominal aorta. Angiography revealed severe suprarenal hypoplasia of the abdominal aorta with bilateral renal artery atresia. Medical support was withdrawn, and the patient died shortly thereafter.