Complement is an important element of immune system playing vital roles in immune surveillance and tissue homeostasis. Increasing evidence shows that complement activations not only protect the body, but contribute to the occurrence and development of autoimmune diseases. Inappropriate activations of complement may also lead to or aggravate various neuro-immunological diseases, including neuromyelitis optica spectrum disease, myasthenia gravis and Guillain-Barré syndrome. Several complement-targeted therapeutics have emerged in recent years, established themselves as popular clinical research topics associated with various autoimmune diseases. Following that note, this review introduces mechanisms of complements participating in autoimmune diseases of the nervous system and the latest development of complement-targeted therapeutics, with the view to contributing to the development of this emerging therapeutic field.