The objective of this study was to determine whether there are age-related variations in the clinical presentation of cerebral arterial ischemic stroke in children after the perinatal period. We performed a retrospective record review of 76 children with cerebral arterial ischemic stroke occurring between 44 weeks of conceptional age and age 19 years. These children were seen by our pediatric stroke group between September 1, 1989 and August 1, 2005. We examined the following clinical presentations: epileptic seizures, focal weakness, and altered mental status. We compared the frequency of each presentation in children with arterial ischemic stroke up to and after age 1 year. Children aged <1 year were significantly more likely than older children to present with epileptic seizures (45.5% vs 10.8%, P = 0.01) and altered mental status (36.4% vs 7.7%, P = 0.02), and there was a trend for them to be less likely than older children to present with focal weakness (45.5% vs 76.9%, P = 0.06). Children aged <1 year with cerebral arterial ischemic stroke were more likely to present with epileptic seizures and altered mental status than children aged >or=1 year, and may be less likely to present with focal weakness. These findings may aid in the recognition of stroke in young children.