Schistosomiasis or bilharzia is a widespread parasitic disease caused by blood flukes of the genus Schistosoma. Some factors have been investigated previously regarding their effect on the pathophysiological mechanism of human schistosomiasis, but the possible influence of the ABO blood group on the severity of Schistosoma infection has been the most promising. Hence, we performed a systematic review and meta-analysis to further investigate the association of the ABO blood group with schistosomiasis susceptibility. Selected publications were retrieved from PubMed up to 21 August 2018, for related studies written in English. Number of cases (with schistosomiasis) and controls (without schistosomiasis) were extracted across all ABO blood types. Odds ratios (OR) and 95% confidence intervals (CI) were computed, pooled and interpreted. Subgroup analysis by the species of Schistosoma infecting the population and the participants’ ethnicity was also performed. The overall analysis revealed heterogeneity in the outcomes, which warranted the identification of the cause using the Galbraith plot. Post-outlier outcomes of the pooled ORs show that individuals who are not blood type O are more susceptible (OR: 1.40; 95% CI: 1.17–1.67; PA < 0.001) to schistosomiasis than those who are blood type O (OR: 0.71; 95% CI: 0.60–0.85; PA < 0.001). Subgroup analysis yielded the same observations regardless of the species of schistosome and the ethnicity of the participants. Results of this meta-analysis suggest that individuals who are blood type B and A are more susceptible to schistosomiasis than those who are blood type O. However, more studies are needed to confirm our claims.