Spinal cord infarction (SCI) causes acute spinal cord dysfunction with high morbidity. Without an inciting event such as a surgical procedure, a definitive diagnosis may be challenging. Thus, patients with a spontaneous (i e, non-traumatic, non-surgical) SCI are often misdiagnosed and the radiological distinction between SCI and other conditions with similar symptoms is more difficult than in cerebral infarction. Compared to cerebral infarction, SCI is rare and only accounts for approximately 1.2% of all strokes. SCI is usually localized to the anterior spinal artery area, causing the anterior spinal artery syndrome. Misdiagnosis may lead to unnecessary and possibly deleterious treatments as well as missed secondary stroke prevention. In this review, a typical case, an overview of the disease and newly proposed diagnostic criteria are presented.