Recognizing childhood arterial ischemic stroke risk factors and presentation are extremely important for a prompt diagnosis and administration of hyperacute treatment requiring a high level of clinical suspicion. Stroke presentation in the pediatric population is not very specific, yet it is imperative to distinguish it from other mimics that may require different medical management. Vascular imaging studies, including MRA, CTA, and advanced neuroimaging techniques (eg, arterial wall imaging and CT/MR perfusion images) are extremely useful in the diagnosis of arterial ischemic stroke. This article offers a review of arterial pediatric ischemic stroke risk factors, clinical presentation, and the imaging appearance of the most common arterial ischemic stroke etiologies.Learning Objective: To recognize multiple risk factors, etiologies, and imaging presentations of pediatric arterial ischemic strokes.