Study design: Case report. Objective: To describe a rare case of congenital absence of the internal carotid artery (ICA) in the setting of acute spinal cord injury and review the existing literature. Setting: Regional Spinal Cord Injury Center of the Delaware Valley, Philadelphia, PA, USA. Clinical presentation: We report on a 47-year-old male who presented as a T2 ASIA A spinal cord injury with a T2-T3 fracture dislocation injury after a 25-feet fall from a tree. Magnetic resonance angiography imaging of the neck carried out as per routine spinal trauma protocol suggested an acute traumatic injury to the carotid artery. Therefore, computed tomographic angiography imaging of the neck was performed, which revealed absence of the left ICA and a diminutive left carotid canal at the skull base, suggesting congenital absence. Follow-up magnetic resonance imaging of the brain to evaluate for acute infarct showed no diffusion restriction contributing to the evidence that this was a congenital anomaly and not traumatic occlusion of the artery. Conclusion: Congenital absence or hypoplasia of the ICA is a rare anomaly. It should be considered in the differential in patients who have absent or diminished carotid arteries on radiologic imaging in the setting of trauma. Spinal Cord (2010) 48, 352-354; doi: 10.1038/sc.2009.125; published online 22 September 2009