Autoimmune encephalitis (AE) is a relatively new category of immune-mediated disease involving the central nervous system. AE-related antibodies can be divided into two groups: antibodies targeting intracellular neuronal antigens and antibodies targeting cell-surface neuronal antigens. Central nervous system idiopathic inflammatorydemyelinating diseases (CNS-IIDDs) is an immune-related group of inflammatory demyelination diseases especially occurring in the brain (including the optic nerve) and/or spinal cord. It is related to several autoantibodies (including anti-aquaporin 4 antibodies and anti-myelin oligodendrocyte glycoprotein antibodies). AE may co-exist with CNS-IIDDs. In this paper, the characteristics of AE, CNS-IIDDs and AE co-existing with CNS-IIDDs were reviewed in combination with relevant literature, in order to provide relevant diagnosis and treatment recommendations for clinicians. However, more high-quality studies are needed to confirm its pathogenesis and clinical features.