Stroke is an important cause of mortality and chronic morbidity in infants and children. Case definitions for perinatal stroke have varied among studies by clinical and laboratory criteria. A recent US National Institutes of Health workshop on perinatal stroke provided consensus recommendations on the definition and classification of perinatal stroke. The incidence of perinatal stroke has been estimated at 1 in 1600 to 5000 births. The clinical presentation of perinatal stroke depends on the time of diagnosis, acute or delayed, but most will present with seizures. Risk factors for perinatal stroke have not been well studied. Several maternal and neonatal disorders have been reported in infants with perinatal stroke. Children who suffer perinatal stroke typically develop long-term disabilities including motor deficits, cognitive and behavioral disorders, and epilepsy. More than half will develop long-term motor or cognitive problems and the recurrence rate after perinatal stroke is very low.