Extracorporeal membrane oxygenation (ECMO) is a frequently used mechanical cardiopulmonary support for rescuing critically ill patients for whom conventional medical therapies have failed. However, ECMO is associated with several complications, such as acute kidney injury, hemorrhage, thromboembolism, and acute brain injury (ABI). Among these, ABI, particularly intracranial hemorrhage (ICH) and infarction, is recognized as the primary cause of mortality during ECMO support. Furthermore, survivors often suffer significant long-term morbidities, including neurocognitive impairments, motor disturbances, and behavioral problems. This review provides a comprehensive overview of the different subtypes of ECMO-related ABI and the updated advance mechanisms, which could be helpful for the early diagnosis and potential neuromonitoring of ECMO-related ABI.