Conditions associated with arterial ischemic stroke (AIS) in children include congenital heart malformations, sickle cell disease, and meningitis, although around half of all cases are cryptogenic. Up to 80% of children with ischemic stroke have cerebrovascular disease, and case control studies demonstrate an association of arterial ischemic stroke in children with hereditary prothrombotic risk factors and infections such as Varicella. Conventional risk factors, such as hypertension and dyslipidemia, may also play a role and most children have several potential triggers rather than a single cause. Treatment recommendations are based on small case series or have been adapted from adult stroke studies; there are no evidence-based data on efficacy in children. Low-dose aspirin appears to be relatively safe. Anticoagulation with heparins, for example, low-molecular-weight heparin or warfarin, may be indicated in children with cardioembolic stroke, arterial dissection, or persistent hypercoagulable states, and blood transfusion has a role in patients with sickle cell disease. Tissue plasminogen activator has been used in a few patients within 3 hours of the onset of symptoms. At present, the benefit of treatment has to be weighed against the risk for each patient, but randomized controlled trials for primary prevention, acute treatment, and secondary prevention of pediatric ischemic stroke are urgently needed.