Abstract: Zika virus-associated Guillain-Barré syndrome (ZIKV-GBS) is an autoimmune peripheral neuropathy and one of the neurological sequelae that occurs after the outbreak of Zika virus (ZIKV). Although the clinical manifestations of ZIKV-GBS mostly follow the classic manifestations of GBS, the pathogenic mechanism, severity and outcome of the pathogens are also different from those of classic GBS. With the widespread of ZIKV worldwide, the incidence of ZIKV-GBS has also increased dramatically. However, there is still a lack of therapeutic (antiviral) or preventive (vaccine) interventions against ZIKV. Therefore, further understanding of ZIKV and ZIKV-GBS for the prevention and treatment of ZIKV- GBS is essential. This article reviews the structural characteristics of ZIKV, the clinical characteristics of ZIKV-GBS, the research status and progress of animal model studies and pathogenic mechanisms, so as to provide new insights and understandings for the occurrence and progression of this disease, and new strategies for treatment and intervention of ZIKV-GBS.