Perinatal arterial ischaemic stroke (PAIS) is increasingly recognised as an important cause of neurological morbidity in children. The aetiology remains unclear although perinatal risk factors have been identified from limited case series. Risk factors for PAIS in term infants are different from those in preterm infants. Maternal primiparity, infertility, cocaine use, prothrombotic disorders, prolonged rupture of membranes, abnormal cardiotocograph, instrumental deliveries and emergency caesarean sections are reported risk factors in term infants. Uncomplicated vaginal delivery and prelabour caesarean section are uncommon in cases of PAIS. The presence of multiple risk factors increases the odds of developing PAIS. For preterm babies, fetal heart abnormalities, twin-twin transfusion and hypoglycaemia are recognised risk factors. Larger cohort studies are required to elucidate further the multifactorial pathway to perinatal arterial stroke.