Perinatal and early childhood stroke are relatively frequent occurrences in Western populations compared with a number of more well publicised diseases of childhood. They have devastating consequences for the child and their family. Despite this, little remains known about the aetiology, pathophysiology and outcome of perinatal and childhood stroke. This review describes the epidemiology, known risk factors, clinical presentation and management of stroke in neonates and children. In particular, we focus on the mechanisms of stroke and the associations with different clinical conditions. There is an urgent need for coordinated research into perinatal and childhood stroke and the recently formed International Paediatric Stroke Study group (IPSS) will hopefully contribute much to improving our understanding and management of these children.