The persistence of embryonic cerebral vessels in the adult is not a frequent occurrence, neither is the presence of multifocal arteriovenous malformations (AVMs) of the brain. The most commonly reported type of persistent carotid-basilar anastomosis is the primitive trigeminal artery, followed by the primitive hypoglossal artery (PHA). In this report, a 30-year-old, right-handed woman hospitalized because of subarachnoid hemorrhage and harboring an intracerebral-intraventricular hematoma resulting from the rupture of one of two independent AVMs of the left cerebral hemisphere, was found also to have a right persistent PHA. One AVM was intraventricular and had ruptured; the other was subcortical, intact in the parietal lobe. The PHA originated as a large anomalous branch of the right internal carotid artery in the neck and joined the basilar artery after entering the posterior fossa through the ipsilateral anterior condyloid foramen, which was enlarged. At craniotomy, the two AVMs were successfully excised with the aid of microsurgical technique. These two independently rare conditions, namely, multifocal cerebral AVMs and persistent PHA, warrant our desire to report this case.