Agenesis of the internal carotid artery (ICA) is a rare congenital entity. This anomaly is typically occult in nature. However, the effects of an incidental discovery secondary to a contralateral ICA aneurysmal rupture can be devastating. The association between agenesis of the ICA and contralateral intracranial aneurysm formation is significantly higher than de novo incidental intracranial aneurysms in the general population. It is important to evaluate the presence of a contralateral intracranial aneurysm in the setting of known agenesis of the ICA. This allows for the performance of prophylactic embolization and characterizes collateral cerebral circulation.