Background: This systematic literature review of the epidemiology of Guillain-Barré syndrome (GBS) identifies trends in incidence rates by age, study method and cause of disease. It is important to have a reliable estimate of incidence to determine and investigate any changes: no previous systematic reviews of GBS have been found. Methods: After critical assessment of the reliability of the reported data, incidence rates were extracted from all relevant papers published between 1980 and 2008, identified through searches of Medline, Embase and Science Direct. Results: Sixty-three papers were included in this review; these studies were prospective, retrospective reviews of medical records or retrospective database studies. Ten studies reported on the incidence in children (0–15 years old), and found the annual incidence to be between 0.34 and 1.34/100,000. Most studies investigated populations in Europe and North America and reported similar annual incidence rates, i.e. between 0.84 and 1.91/100,000. A decrease in incidence over the time between the 1980s and 1990s was found. Up to 70% of cases of GBS were caused by antecedent infections. Conclusions: Our best estimate of the overall incidence of GBS was between 1.1/100,000/year and 1.8/100,000/year. The incidence of GBS increased with age after 50 years from 1.7/100,000/year to 3.3/100,000/year.