We report a case of symptomatic symmetrical, bilateral absence of the cavernous internal carotid (ICA) and transdural vertebral artery (VA) segments with formation of a rete mirabile. There have been similar reports in Asian patients; ours is the first in the Western population. A 29- year-old woman presented with recurrent temporary blindness and an episode of minor subarachnoid haemorrhage. Angiography demonstrated absence of the C5 and C6 segments of the ICA and the C1/2 segments of the VA bilaterally, with typical carotid rete mirabile formation to reconstitute the distal flow. Comparative anatomy and embryology show retia in several species and point to their secondary origin. The anomalies of the ICA cannot be called agenesis but rather absence since they are likely to result from perinatal disappearance of the artery rather its failure to develop.