Acute ischemic stroke (AIS) is a neurological emergency, and early treatment may improve symptoms and relieve ischemic brain injury. Some patients present with stroke-like symptoms, however the actual medical conditions are considered as“stroke mimics”. With similar clinical characteristics to true strokes, SM has been proved to include a series of etiologies and conditions. Although SM could be evaluated and rapidly recognized by imaging and clinical symptoms, some patients may receive inappropriate acute treatments, which brings certain drug treatment risks and emergency department crowding. Therefore, early and correctly identification of SM is essential for AIS to avoid potential risks of using AIS drugs and the delay in primary treatment. Multimodal imaging protocols and prediction scales based on clinical features can help neurologists identify SM in emergency situations. This review summarizes the prevalence of SM, the distribution of disorders spectrum, clinical features, safety of thrombolytic therapy, and differential diagnosis and prediction tools for SM.