Background: Pulmonary embolism (PE) remains a major cause of cardiovascular morbidity and mortality. Classical risk factors explain only part of the interindividual variability in thrombotic risk. Non-O blood groups are associated with higher plasma levels of von Willebrand factor and factor VIII, suggesting a potential prothrombotic mechanism. This systematic review and limited meta-analysis examined the relationship between ABO blood group and PE risk. Methods: Following PRISMA 2020 guidelines, PubMed, Embase, and Web of Science were searched through August 2025 for observational studies reporting ABO blood group and objectively confirmed PE in adults. Eligible designs included cohort, case–control, and registry-based studies. Two reviewers independently extracted data and assessed risk of bias using the Newcastle–Ottawa Scale. Comparable effect estimates were pooled using a random-effects model (DerSimonian–Laird method, inverse-variance weighting). Results: Four studies met inclusion criteria, comprising more than 300,000 participants from North America, Europe, and Asia. All reported a higher incidence of PE among non-O compared with O blood groups. Pooled analysis of two large population-based cohorts yielded a summary relative risk of 1.36 (95% CI 1.20–1.54; I2 = 2.6%), indicating a modest but consistent association. Data on recurrence, severity, and mortality were limited and heterogeneous. Conclusions: Non-O blood groups are associated with an approximately 30–40% higher risk of PE across diverse populations. While evidence is insufficient for causal inference, ABO phenotype represents a biologically plausible and readily available marker that may complement multifactorial models of thromboembolic risk.