Abstract: Objective To investigate the relationship of single nucleotide polymorphism (4G/5G) in the promoter of plasminogen activator inhibitor-1 (PAI-1) and plasma PAI-1 level with gastrointestinal bleeding in Henoch-Schönlein purpura (HSP). Methods A total of 524 children with HSP in acute phase were recruited, and divided into gastrointestinal bleeding group (bleeding group, n = 186) and non-gastrointestinal bleeding group (control group, n = 338). The genotype frequency of 4G/5G polymorphism, the plasma PAI-1 level, and other parameters related to coagulation and fibrinolysis were measured and compared between two groups. Results The levels of platelet count (PLT), platelet distribution width (PDW), serum D dimer (DD), serum PAI-1 were significantly higher in the bleeding group than those in the control group, and the levels of mean platelet volume (MPV) and plasma fibronectin protein of fibrinogen (FIB) were significantly lower in the bleeding group than those in the control group (P < 0.05). The genotype frequency of 4G/4G was significantly higher in the bleeding group than that in the control group (P = 0.044). The plasma PAI-1 level and DD level was high in 4G/4G genotype. Conclusions The gene polymorphism of PAI-1 4G/5G may affect the pathological process of gastrointestinal bleeding in HSP by influencing the expression of PAI-1 and other factors related to coagulation and fibrinolysis systems.