To study and discuss the clinical characteristic, treatment and prognosis of anti-MOG antibody associated demyelinating disease. Methods: A rare case of anti-MOG antibody associated encephalitis was reported and the related literature was reviewed. Results: Epilepsy was the first clinical symptom in this case. MRI showed involvement of cerebral cortex, deep gray matter and pia mater. The patient was positive for MOG antibody in serum and cerebrospinal fluid. The symptoms were relieved obviously after corticosteroid treatment. Searching for previous cases of MOG antibody encephalomyelitis, there were 61 adult patients with encephalitis. The most common clinical features were ADEM and brainstem encephalitis. At present, it is considered that the effective treatment is immunotherapy. Conclusion: The mechanism of MOG antibody encephalomyelitis is not clear, and the clinical features, treatment and prognosis are uncertain. Therefore, early identification and timely treatment are important factors for better prognosis.