Aortic thrombosis of the newborn is a rare condition associated with poor functional and vital prognosis. Most cases are related to umbilical arterial catheterization, but other risk factors include sepsis, extreme prematurity, asphyxia and prothrombotic conditions. We present a case of neonatal aortic thrombosis presenting with a lower limb ischemia occurring at the second week after birth in association with respiratory distress. Its imaging features are displayed along with a literature review. The Doppler ultrasonography made the initial diagnosis and Contrast Enhanced Computed Tomography provided precise location and extension of the thrombus. In Conclusion, Doppler Ultrasonography is first-line examination for diagnosis of aortic thrombosis in neonates. However, its lack of anatomic precision makes the Contrast Enhanced Computed tomography the imaging technique of choice in investigation of ischemia due to aortic thrombosis, as it provides the topography and extension of the thrombosis, also identifies the ischemic phenomena of abdominal organs and limbs.