Increasingly distinct patterns of focal ischemic injury in the fetal and perinatal brain are recognized. Improved classification has afforded advances in risk factor identification, pathophysiology hypotheses, outcome prediction, and potential avenues for intervention. Cerebrovascular occlusion leading to perinatal stroke may be arterial or venous, symptomatic or subclinical, and it can occur across multiple time frames. Distinguishing causative factors from mere associations represents a major challenge with important implications for studies of pathogenesis. The adverse outcomes suffered by most children highlight the need for further research. Reviewed here are the current understandings, recent advancements, and future directions for research in perinatal ischemic stroke.