Objectives Bilateral renal agenesis is a rare congenital anomaly that is associated with high neonatal mortality. Bilateral renal agenesis is most often present with anhydramniosis in the mid-trimester. Case presentation We report a case of bilateral renal agenesis diagnosed prenatally. We presented the ultrasound and pathology images of this fetus with a new sonographic sign, segmental anterior deviation in the abdominal aorta. Conclusions To our knowledge, this is the first reported case of a fetus with a segmental aortic anterior deviation.