A retrospective population-based study was conducted to calculate incidence of pediatric stroke, detect risk factors, determine long term outcome and to optimize therapy. Patients (age: >1 month and <19 years) with stroke in Vorarlberg, Austria, from 1984-2005 were investigated. Outcome was evaluated in terms of neurological deficits, neuropsychological measures, and quality of life. Consequences on therapy were established according to current guidelines. Twenty-two children, median age 6 years (range, 0.9-14) years, 16 ischemic and 6 hemorrhagic stroke, correspond to an incidence of 1.96 and 0.74/100.000 child-years, respectively. No child died; one had a recurrent stroke. Twenty children (12 boys) were included after a median time of 3.7 years (range, 0.4-18). Risk factors were vasculopathy (17/20; 85%), lipometabolic disorders (17/20; 85%), and prothrombotic abnormality (10/20; 50%). Three children had no risk factor; four children had a reduced quality of life. The study led to therapeutic consequences in 13 of 20 children (65%): aspirin (5/20; 25%), folic acid in (3/20; 15%), and rehabilitative therapy (9/20; 45%). Most children with pediatric stroke have more than one risk factor, mainly vasculopathies or elevation of lipoprotein(a). Overall outcome is determined solely by neurological deficits and is positively influenced by good quality of life.