Impairment of the fibrinolytic system is one of the most sensitive markers of endothelial dysfunction, and a major risk factor for future cardiovascular disease in adults. Persistent endothelial dysfunction, which is an early pathophysiological event in atherogenesis, occurs in some patients following Kawasaki disease (KD). The aim of this study was to assess whether impaired fibrinolysis is present in long-term survivors of KD. The study included 42 children with a documented history of KD presenting without coronary lesions (n = 22), with resolved coronary aneurysms (n = 13), or with persistent giant aneurysms (n = 7), and 26 healthy age-matched teenagers as controls. Blood samples were collected from all patients and controls prior to and following venous occlusion (VO) stress testing, and assayed for tissue plasminogen activator (tPA), plasminogen activator inhibitor (PAI)-1, plasminogen, alpha2-antiplasmin (a2-AP), alpha2-macroglobulin (a2-M), D-Dimer, fibrinogen, and von Willebrand factor (vWF). Significantly decreased fibrinolytic activity following VO was detected in patients compared to controls due to decreased tPA antigen, and increased PAI-1 activity. In addition, patients had significantly increased plasma concentrations of plasminogen and fibrinogen, which were related to similar increases of a2-M compared to controls. Decreased fibrinolytic activity was found in patients with coronary aneurysms but also in those without coronary lesions. In summary, a decreased fibrinolytic activity reflects persistent endothelial damage following acute KD, potentially predisposing these patients to accelerated atherosclerosis and cardiovascular disease in early adult life.