Stroke is the second leading cause of death worldwide (Go et al. Circulation 129:e28-292, 2014) and is a major cause of morbidity and mortality. Compared with older adults, arterial ischemic stroke (AIS) is relatively uncommon in children and young adults, comprising 5-10 % of all stroke (Biller Nat Rev Cardiol 6:395-97, 2009), but is associated with significant cost. In contrast to the declining overall incidence of stroke, some early studies suggest that the rate of stroke hospitalizations in children and young adults is rising (George et al. Ann Neurol 70:713-21, 2011; Kissela et al. Stroke 41:e224, 2010; Nguyen-Huynh et al. Stroke 43, 2012), emphasizing the importance of understanding the similarities and differences in etiology and treatment of AIS across the age spectrum. Among the most common causes of AIS in children are cardioembolism (often related to congenital heart disease), cervicocephalic arterial dissections, focal arteriopathy of childhood and several genetic and metabolic disorders, such as sickle cell disease (SCD). AIS in young adults is less well understood, but likely overlaps in etiology with both children and older adults. Young adults with AIS often have classic atherosclerotic risk factors similar to older adults, but are also more likely to have thrombophilias, cervicocephalic arterial dissections and cardioembolism, similar to children with AIS. Since little evidence exists regarding both acute treatment and secondary prevention after AIS in children and young adults, standard treatment practices are mainly extrapolated from research done in older adults. In most cases we recommend treating young adults per the guidelines published by the American Heart Association for adults with stroke (Jauch et al. Stroke 44:870-947, 2013; Kernan et al. Stroke 45:2160-2236, 2014) and children per the equivalent guidelines regarding pediatric stroke (Roach et al. Stroke 39:2644-91, 2008). It is also important in children and young adults to consider less common structural, metabolic and genetic risk factors for stroke, which may require more specific treatment. Other standard risk factors for stroke, including hypertension, hyperlipidemia and diabetes mellitus should also be addressed, but are less likely in children and young adults. Given the lack of data and possibility of rare underlying etiologies such as Antiphospholipid Antibody Syndrome or Ehlers-Danlos syndrome, we recommend including multiple specialists in the care of these patients, such as hematologists and vascular neurologists.