This review will discuss important developments in childhood arterial ischemic stroke over the past decade, focusing on improved understanding of the causes, consequences, and targets for intervention. Risk factors for childhood arterial ischemic stroke are different to adults. Infections, particularly herpes group viruses, are important precipitants for stroke. Non-atherosclerotic arteriopathies are the most common cause of childhood arterial ischemic stroke and an important predictor of recurrent events. Recent advances include the identification of serum biomarkers for inflammation and endothelial injury, and imaging biomarkers to monitor for vascular progression. Multicenter trials of immunotherapies in focal cerebral arteriopathies are currently in development. Recognition of clinical and radiological phenotypic patterns has facilitated the discovery of multisystem disorders associated with arterial ischemic stroke including ACTA2 arteriopathy and adenosine deaminase 2 deficiency. Identification of these Mendelian disorders provide insights into genetic mechanisms of disease and have implications for medical and surgical management. In contrast to adults, there are long diagnostic delays in childhood arterial ischemic stroke. Refinement of pediatric Code Stroke protocols and clinical decision support tools are essential to improve diagnostic certainty and improve access to reperfusion therapies. Children do not recover better than adults following arterial ischemic stroke, with more than half of survivors having long-term impairments. The physical, cognitive, and behavioral consequences of childhood arterial ischemic stroke are increasingly reported but further research is required to understand their impact on participation, quality of life, psychosocial, and family functioning. Longitudinal studies and the use of advanced imaging techniques, to understand neurobiological correlates of functional reorganization, are essential to developing targeted intervention strategies to facilitate recovery.