Purpose or Case Report: Congenital absence of the internal carotid artery (ICA) is a rare vascular anomaly, with a prevalence below 0.01%. This case report presents an unusual instance of idiopathic intracranial hypertension (IIH) and migraine, for which the workup revealed the unexpected absence of the bilateral ICAs.

A 13-year-old female, previously healthy, experienced severe, persistent bilateral occipital headaches, neck pain, blurry vision, photophobia, and visual aura. Ophthalmological findings showed optic disc swelling, particularly in the right eye. Pediatric Neurology assessed the patient, finding papilledema (predominantly in the right eye) with no other neurological deficits.

Lumbar puncture yielded an elevated opening pressure (34 cm H2O). MRI brain demonstrated imaging characteristics indicating IIH. MRV head/neck excluded dural venous sinus narrowing or thrombosis. MRA head/neck revealed congenital bilateral ICA absence. Both vertebral arteries were patent, and the basilar artery supplied the anterior cerebral circulation.

The patient was diagnosed with IIH and migraine. She was discharged with Diamox and Topamax, with ongoing management by pediatric neurology.

This case report will review the clinical background and the advanced imaging findings associated with an uncommon intracranial vascular anomaly in pediatric neurology, including arterial spin labeling (ASL). Bilateral ICA absence, though exceptionally rare, can contribute to neurological manifestations, including IIH and migraine.

Co-occurring IIH and migraine, alongside congenital bilateral ICA absence, pose a unique diagnostic challenge. A multidisciplinary approach and heightened awareness among healthcare providers are essential for managing such complex presentations.
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