Neuromyelitis optica spectrum disorders (NMOSD) is a kind of inflammatory demyelinating disease of central nervous system. It has high recurrence rate, high disability rate and poor prognosis. Studies have shown that the novel markers of NMOSD help to predict the severity and clinical onset of the disease and monitor the the rapeutic effect. In this review, literature on novel markers of NMOSD reported in recent studies was reviewed. Clinical detection of specific markers may help to diagnose and treat NMOSD earlier and improve the prognosis of patients with NMOSD.