Acute ischemic stroke is rare in children and is associated with heart, hematologic, and vascular conditions. A high index of suspicion is required for proper diagnosis and intervention. We report a case of an acute ischemic stroke in a 13-year-old girl with a past medical history of left subscapular arteriovenous malformation. She started complaining of fever and nasal obstruction four days before admission, followed by a brief episode of blurred vision and vomiting two days later, as well as a headache on the day of admission. Facial asymmetry and slowed speech started at admission, and examination revealed right hemiparesis with homolateral facial involvement and ataxia. Magnetic resonance imaging confirmed a left pons ischemic lesion, and we started anticoagulation / anti-aggregation treatment. Angio-magnetic resonance revealed occlusion of the basilar artery and narrowing of the right vertebral artery. A complete resolution of basilar artery occlusion was noted after 40 days, while narrowing of the vertebral artery persisted. We discuss factors contributing to acute ischemic stroke and strategies to prevent future events.