The clinical presentation, risk factors, causes, vital or functional prognosis, and acute management options for stroke occurring in neonates and children are specific, differing from those observed in young adults. Compared with the adult population, less is known about the epidemiology of stroke in the under-18 population where the disease could become more frequent because of advances in both neonatal resuscitation techniques for cerebral disorders and neuroimaging techniques enabling the diagnosis of small lesions. Clinical features are often delayed, especially in neonates, and unlike epilepsy or dystonia of the affected limb, which are frequent complications, aphasia is rather rare. The most frequent causes of stroke at the beginning of life are cardiac embolism, for ischemic stroke, and arteriovenous malformations, for intracerebral hemorrhage. Acute management at this age is specific. This article reviews the literature on the epidemiological and clinical features, the main causes, and the acute management guidelines of stroke occurring in newborn infants and children and highlights the need for neurologists to have comprehensive knowledge of this disease.