Helicobacter pylorihas been proposed as a possible etiologic factor of ocular adnexa lymphoma (OAL), although with conflicting results. To assess the involvement ofH. pyloriin OAL, as (1)H. pyloriDNA positivity on OAL specimens, and (2) prevalence ofH. pylorigastric infection in patients with OAL. A systematic review of studies assessingH. pyloriin patients with OAL was conducted by searching electronic databases from their inception to May 2019. Pooled positivity forH. pyloriin OAL specimens detected by polymerase chain reaction, and pooled prevalence ofH. pylorigastric infection, were calculated with 95% confidence interval (CI). Eleven studies with 308 patients were included. Pooled positivity forH. pyloriwas 16.8% in all OALs and 22.7% in MALT OAL, with high heterogeneity among studies. Pooled prevalence ofH. pylorigastric infection in patients with OAL was 34.7%, with low statistical heterogeneity. In conclusion,H. pyloriseems to be involved in a subset of OAL, but the heterogeneity found needs to be investigated in further studies. The prevalence ofH. pylorigastric infection in patients with OAL does not seem to differ from that of the general population.