Background: Infrarenal aortic agenesis is an extremely rare condition that has been described in a few case reports in the literature. Imaging clues to distinguish infrarenal abdominal aorta agenesis from other abdominal aortic occlusions include: the absence of an aorta at the infrarenal level without peri-aortic soft tissue or thickening of the aortic wall that suggests vasculitis or mass, and collaterals, such as a Riolan arch, for the anomaly to be life reconcilable. Case Presentation: In this case report, a 60-year-old female patient with a diagnosis of infrarenal segmental aorta agenesis is described. Computed Tomography (CT) angiography was performed to evaluate the vascular involvement of the patient who is followed up with sarcoidosis. It was observed that the abdominal aorta terminated abruptly at the level of the renal arteries, and refilled via collaterals 2 cm distal to this level. Conclusion: Recognition of this very rare entity by the radiologist is important in guiding the clinician in terms of treatment follow-up protocol. It is aimed to make this entity easier to recognize by radiologists with this case report.

Keywords: Abdominal aorta agenesis, volume-rendered computed tomography, Riolan arch, collaterals.