Perinatal stroke encompasses six classical disease-states with diverse causality, mechanism, time of onset, mode of presentation and outcome. By definition, neonatal stroke – divided into neonatal arterial ischemic stroke, neonatal cerebral sino-venous thrombosis and neonatal primary haemorrhagic stroke – manifests in the first 28 days of life with seizures or others features of neonatal encephalopathy: apnoea, lethargy, hypotonia, bulging fontanelle… In contrast, for children with presumed perinatal (arterial or venous) ischemic or haemorrhagic stroke, i.e. whose onset is undetermined, signs become apparent only beyond the neonatal period, most often with motor asymmetry, milestones delay or seizures. Acute or remote MRI defines the type of stroke. Growing evidence suggests that, on the one hand presumed perinatal arterial ischemic stroke and neonatal arterial ischemic stroke, and on the other hand presumed perinatal haemorrhagic stroke and neonatal haemorrhagic stroke, represent the same pathophysiological entities and differ only by the timing of clinical presentation or diagnosis. Eventually, this would reduce to only four main categories of perinatal stroke. Except in some specific and well-identified circumstances (meningitis, cardiopathy, haemorrhagic diathesis…), the specific mechanism of stroke is unknown. However, many determinants are recognized and common among the diverse categories of perinatal stroke (and neonatal conditions/diseases as a whole): first pregnancy, chorioamnionitis, perpartum asphyxia… accounting for a multifactorial aetiology. Nevertheless, the final pathway leading to stroke remains largely undefined. Acute care relies on homeostatic maintenance. Seizures are often self-limited and antiepileptic drugs shoul be discontinued before discharge. Anticoagulation for a few weeks is an option in some cases of sino-venous thrombosis. Many children develop mild to moderate multimodal developmental issues which require a multidisciplinary approach.