Purpose: Aortic arch anatomical variations are of surgical importance. Many variations of the aortic arch and associated vessels may be found incidentally or post-mortem.

Methods: A 61-year-old presented with symptoms suspicious of a cerebrovascular accident.

Results: The patient was found to have the right internal and external carotid arteries emanating from the right subclavian artery in absence of a common carotid artery on computed tomography angiography of the neck.

Conclusion: A recent comprehensive review noted 41 published cases of common carotid artery aplasia with the right internal carotid originating from the subclavian artery in only 4 of these cases. The current case represents a rare absence of the right CCA with the right ICA and ECA originating separately from the right subclavian artery.