| Summary: | Congenital absence of the internal carotid artery (ICA) is a rare congenital developmental anomaly, which is often associated with carotid canal atresia and structural abnormalities of the ICA. It may be asymptomatic, or shows noticeable symptoms such as Horner syndrome, loss of visual field, trigeminal neuralgia, pulsing tinnitus, memory dysfunction/dementia, transient ischemic attack, intracranial aneurysm, subarachnoid hemorrhage, pituitary dysfunction, and developmental malformations in multiple organs. We reported a case of congenital absence of the left ICA with Horner syndrome, aneurysm, and secondary Parkinson's syndrome. Along with the retrospective analysis of diagnosis, treatment and disease progression of this case, a review of relevant literature was conducted, to improve the recognition of this rare complication.
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