In term infants there has been an increasing awareness of stroke as part of the differential diagnosis of neonatal seizures and other neurological symptoms. The etiological factors responsible for perinatal cerebral infarction, the clinical presentation, and long-term consequences are diverse. Most perinatal arterial ischemic stroke studies looking at maternal, perinatal, and infant characteristics exclude preterm infants from the population studied. More than 10 years ago, it had already been described that perinatal stroke is not only a disorder of term or near-term infants, however, data about preterm infants suffering from stroke are scarce. It is not clear whether this is due to a lower incidence, a lack of awareness, or a different (sub)clinical presentation. In this review the differences between preterm and full-term infants in incidence, clinical presentation, neuro-imaging findings, vascular distribution, and risk factors are described.