With the epidemiology now well described, stroke has been uncovered as a frequent cause of neurological disease in children. Arterial ischemic stroke (AIS) is the most common type, affecting both neonates and older children. The number of associated risk factors is large with current efforts directed toward understanding the mechanisms by which they translate into a stroke. Advanced neuroimaging modalities are improving diagnostic accuracy while affording unique avenues to explore the pathophysiology of stroke and the response of the developing brain to injury. While acute interventions for pediatric stroke are in their infancy, the first randomized controlled clinical trials for the initial management and secondary prevention of pediatric stroke are poised to begin. Outcomes from childhood stroke are well defined though the accurate predictors required to guide treatment and rehabilitation remain elusive. We review the essential background, recent advances, and current clinical research issues pertaining to AIS in children.