Background & Aims: Helicobacter pylori (H. pylori) infection is proposed to be related with autoimmune diseases, such as Graves' Disease (GD). This study aimed to assess the association between H. pylori infection and GD. Methods: A systematic literature review was conducted using Pubmed and Cochrane library. The quality of enrolled studies was assessed by the Critical Appraisal Skills Program Oxford. A fixed-effect model approach was used if there was no heterogeneity; otherwise, a random-effect model was used. Heterogeneity was assessed using I2. Publication bias was assessed by funnel plot. All data were analyzed using REVIEW MANAGER 5.3. Results: Six studies from Europe and Asia involving 983 patients were included. Overall H. pylori infection was significantly associated with GD (OR 2.7; 95% CI: 1.47-4.99; p < 0.001). In subgroup analysis of 3 studies using non-serological diagnostic method, the prevalence rate of H. pylori infection was higher in GD group (78.26% VS 42.42%) with significant relationship (OR 4.93; 95% CI: 3.16-7.69; p < 0.00001; I2 = 0%). The CagA antibody prevalence was significantly higher in GD group (46.57% VS 20.29%; OR 4.41; 95% CI: 2.65-7.33; p < 0.00001; I2 = 56%). No publication bias was observed. Conclusion: Our study showed association between H. pylori infection and GD. It might suggest the need of H. pylori examination in GD patients and the impact of H. pylori eradication in the treatment of GD.