Stroke is a rare, but terrifying and devastating disease in childhood. Over the last two decades, childhood stroke is increasingly being recognized as an important cause of childhood morbidity and ranges among the top 10 causes of death in children. 1-3 Thus childhood stroke is an important burden, not only for affected children and their families, but also for health insurances and socioeconomic reasons. The costs for pediatric stroke are substantial: In an North American study, the average cost of a childhood stroke admission was $81,869, the average adjusted 5 years costs are $135,161. 4 International data suggest an incidence of childhood stroke of 2 to 5/100,000 children per year. 1 Data from the Swiss Neuropediatric Stroke Registry suggest an incidence for arterial ischemic stroke (AIS) in childhood of 2.1/ 100,000 children per year. 5 There is a suggestion of a higher incidence, due to clinically unrecognized events. 6 For yet unknown reasons there is a worldwide male predominance in childhood stroke. 7,8 Many excellent reviews have been published recently. 1,9-12 There are three guidelines for treatment published. 13-15 This article will not attempt to give a review of the subject, but will try to guide a physician through many aspects one has to consider, dealing with a child with acute arterial ischemic stroke, from emergency to the long-term follow-up and giving some inputs on differential diagnosis, work-up, and treatment as linked to the symptoms and Keywords ► childhood stroke ► risk factors ► etiology ► treatment ► manifestation ► outcome