Polymorphism in plasminogen activator inhibitor-1 gene is suggested to be associated with an increased risk of venous thromboembolism. The aim of this study was to investigate the association of plasminogen activator inhibitor-1 gene polymorphism and its coexistence with factor-V-Leiden and prothrombin-20210 mutations in pulmonary thromboembolism. The authors investigated plasminogen activator inhibitor-1 4G/5G polymorphism, factor-V-Leiden, and prothrombin-20210 mutations in 143 pulmonary thromboembolism patients and 181 controls. Plasminogen activator inhibitor-1 4G/4G, 4G/5G, and 5G/5G gene polymorphisms and prothrombin-20210 mutations were not different between cases and controls. Factor-V-Leiden mutation was present in 21.0% and 7.7% of the cases and controls, respectively, P = .001. Neither different plasminogen activator inhibitor-1 genotypes and 4G allele nor coexistence of the allele with factor-V-Leiden or prothrombin-20210 was associated with the risk of recurrence. As a result, plasminogen activator inhibitor-1 gene polymorphism or its concomitant presence with mentioned mutations was not found to be associated with increased risk for pulmonary thromboembolism or recurrent disease in this study.