Abstract
BACKGROUND: Persistent primitive hypoglossal artery (PPHA), a remnant of embryonal circulation, is a rare variant of the posterior cerebral circulation. Seven prior cases of posterior circulation stroke in the setting of PPHA have been described in the literature, with all but one case being attributable to atherosclerotic embolization from the internal carotid artery (ICA) through the PPHA. CASE DESCRIPTION: We report a unique case of a young male with a PPHA presenting with a "top of the basilar" syndrome following the repair of his atrial septal defect who underwent emergent revascularization via endovascular mechanical aspiration thrombectomy. The patient underwent a successful aspiration thrombectomy with significant improvement in his clinical exam. CONCLUSIONS: Considering the rarity of this persistent fetal anastamosis, it is important to be aware of the propensity for unusual presentations in the context of stroke, understand the management of the problem, and expeditiously treat the patient.