Background and objectives The findings on the associations between potential genetic variants and risk of Guillain-Barre syndrome (GBS) are controversial. We conducted a meta-analysis for candidate genes to provide the evidences for the current understanding of the genetic association with GBS. METHODS: We searched relevant studies without language restriction in PubMed, Embase and Cochrane library through May 2011. The strengths of the associations between genetic variants and GBS risk were estimated by odds ratios (ORs) with 95% confidence intervals (CIs). Random-effects models or fixed effects model was applied based on the heterogeneity test. RESULTS: We identified 12 case-control studies involving 1,590 GBS cases and 2,154 controls for the analysis. Because of limited eligible data, our meta-analysis specifically focused on 6 genetic variants of 3 candidate genes, TNF-alpha, FcgammaR and CD1. We found that TNF-alpha 308 G/A polymorphism was significantly associated with the risk of GBS in the overall population (GG+GA vs. AA: OR=0.32, 95%CI=0.16-0.62; GG vs. AA: OR=0.36, 95%CI=0.19-0.68). Subgroup analysis further provided evidence of significant association between TNF-alpha 308 G/A and risk of the GBS in Asian population (GG+GA vs. AA: OR=32, 95%CI=0.11-0.93; GG vs. AA: OR=0.32, 95%CI=0.15-0.68). In addition, we did not observe significant associations between FcgammaRIIA R/H, FcgammaRIIIA F/V, FcgammaRIIIB NA1/NA2, CD1A 1⁄2 and CD1E 1⁄2 polymorphisms and susceptibility for developing GBS. CONCLUSIONS: Our findings showed that TNF-alpha 308A allele might be a moderate risk factor for GBS. However, the results should be interpreted with caution due to the limited number of studies available.