Abstract: Familial Mediterranean fever (FMF) is an autoinflammatory disease caused by mutations of the MEFV gene. The main clinical manifestations are recurrent fever and serous inflammation in various parts of the body. Some patients with FMF may also develop neurological manifestations such as epilepsy, demyelinating disease, aseptic meningitis. As neurological manifestations are rare in patients with FMF, the association between the two is often overlooked in clinical practice. In this paper, we reviewed the neurological manifestations of FMF and their relationship with genotypes, so as to deepen the understanding of the clinical manifestations of FMF and provide help for the diagnosis and treatment of patients with neurological manifestations.