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Arterial ischaemic stroke (AIS) in childhood is a serious disorder about which little is published. The aim of this study is to determine the epidemiology and outcome of AIS in Australian children. Cases of childhood AIS occurring at the Royal Children's Hospital, Melbourne 1993−2001, were identified by medical record search using International Classification of Disease Codes. Information was collected on demographics, risk factors, arterial distribution, results of thrombophilic testing, management and outcome. During the 8 years of review 95 patients presented with 98 cases of AIS calculating an incidence of 1.8 per 100 000 children per year. Children less than 12 months of age represented greater than one third of all cases. Identifiable risk factors were present in 64% of cases with congenital heart disease the major risk factor. Thrombophilic testing was incomplete with initial abnormalities present in 18% of cases tested. The estimated stroke-related mortality was 8.4%. Of the patients who survived and who had follow-up details available, 78% had a neurological deficit. Twenty-six patients (26%) received anticoagulation. There was no statistically significant association between treatment with anticoagulation and normal neurological outcome. AIS is over-represented in children under 12 months of age and results in death or residual neurological impairment in the majority of cases. Further prospective studies are needed to identify risk factors for poor outcome. The recently established Australian and New Zealand Stroke and Thrombophilia Registry should provide important information on clinical and laboratory based risk factors and create a basis for international clinical trials to improve the outcome of childhood AIS. [ABSTRACT FROM AUTHOR]