Abstract
Congenital unilateral agenesis of the internal carotid artery (ICA) is a rare anomaly. Due to proper sufficient collateral circulation via the circle of Willis most cases are asymptomatic, but patients can also present with ischemic or hemorrhagic cerebrovascular insults. The absence of the bony carotid canal is essential to differentiate this anomaly from chronic ICA occlusion. Awareness of this situation by clinicians and radiologists is essential because these patients have an increased incidence of various intracranial pathologies. We report two cases of this rare developmental congenital abnormality occurring in two young patients and describe the presentation, diagnosis, determined developmental causes, imaging findings, and complications.