Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyradiculoneuropathy that affects the peripheral nervous system. The study aimed to describe the incidence of GBS in the world up to the year 2020. A systematic review was conducted. Searches were done in four databases, PUBMED, EMBASE, EBSCO and Biblioteca virtual em Saude (BVS), and in grey literature and manual search in the reference lists of eligible studies. A total of 72 studies were included. The incidence of GBS among the cohort studies varied from 0.30 to 6.08 cases per 100.000 habitants and 0.42 to 6.58 cases per 100.000 person-years. Among the self-controlled studies, the risk incidence ranged from 0.072 to 1 case per 100.000 habitants and 1.73 to 4.30 cases per 100.000 person-years. The reported incidence of GBS in the world among the studies included in the review is slightly higher than that reported in previous studies. The highest incidence rates were associated with public health events of international concern. •Update on the frequency of GBS in the world among WHO regions up to 2020.•First systematic review to evaluate the frequency of Guillain-Barré Syndrome over a long period of time, 1985 -2020.•Update on the frequency of GBS in children and adolescents.•Update on the frequency of GBS variants among the WHO regions.•The study provides a background frequency of GBS before the start of the Covid-19 pandemic.