Ischemic brain injuries are rare in childhood, but can result in irreversible damage and may significantly impact on the quality of life of affected individuals. A significant proportion of ischemic infarctions are caused by primary angiitis of the CNS (PACNS). To make the diagnosis of PACNS, differential diagnoses, including systemic diseases associated with vasculitis, must be ruled out. In analogy to systemic forms of primary vasculitis, PACNS is classified based on the calibre of affected vessels. Diagnosis and classification are based on clinical presentation, laboratory investigations, imaging, as well as brain biopsies in the case of small vessel vasculitis. Due to a lack of prospective and controlled trials, treatment is currently based on small case series and expert recommendations. Available medicinal options include systemic anticoagulation, high-dose corticosteroids, and, where necessary, cyclophosphamide for remission induction, followed by disease-modifying antirheumatic drugs for maintenance treatment. The pathophysiology of PACNS is very incompletely understood. The investigation and identification of genetic causes will result in an updated classification and new targeted and individualised treatment options. To promote this, international collaborations are necessary to overcome issues caused by the rarity of childhood PACNS.