Cerebrovascular disorders are an important cause of mortality and chronic morbidity in children. Ischemic stroke is more common than cerebral venous thrombosis and hemorrhagic stroke in children. Several medical disorders have been associated with stroke in children, and a thorough evaluation of underlying causes is needed to determine the best treatment and prevention strategy. The treatment and prevention of stroke in children is not well studied, and current recommendations are based on adult studies, nonrandomized trials, or expert opinion. Children with stroke require immediate, special attention and if possible should be stabilized and transferred to an institution that can offer pediatric neurovascular expertise and care. All children with stroke should be referred to or have their care managed by a pediatric neurologist. The treatment of stroke in adults is well studied, and when applicable this evidence should be considered in the treatment of children with stroke. Data from animal and adult stroke studies have demonstrated a benefit for the aggressive treatment of infection, fever, blood pressure, hypo/hyperglycemia, intracranial pressure, and seizures, and should be applied to children with stroke. The use of thrombolytic, antithrombotic, and antiplatelet therapies is based on adult studies, cohort studies, and/or expert opinion. Two consensus guidelines regarding the treatment of arterial ischemic stroke and cerebral venous thrombosis were recently published and recommend the use of anticoagulants or antiplatelet agents in the acute setting, depending on the underlying cause of stroke. The evidence for the primary prevention of stroke in children is restricted to sickle cell disease (SCD) and derived from the Stroke Prevention in Sickle Cell Study Project studies. Long-term chronic transfusion therapy to maintain hemoglobin S levels below 30% is indicated in children with SCD and intracranial stenosis. It has also been recently determined that chronic transfusion therapy should not be stopped in children with SCD and an increased risk for stroke. The recurrence rate of arterial ischemic stroke (AIS) in children ranges from 6% to 30% and is highest among children with recurrent transient ischemic attack, cardiac disease, arteriopathies, and metabolic and coagulation abnormalities. Recommendations for secondary prevention are based on adult studies and the underlying pathophysiology of the stroke. Antiplatelet therapy (aspirin 1-5 mg/kg/day) is recommended in most children with a history of AIS. Although there is minimal evidence to support its use in children, anticoagulation may be indicated in AIS associated with extracranial arterial dissection, prothrombotic disorders, cardiac disease, severe intracranial stenosis, and recurrent AIS while on antiplatelet therapy.