Patients with heparin-induced thrombocytopenia have a poor prognosis and high mortality, thus surgical risk under extracorporeal circulation increased. Early diagnosis, safe and effective alternative anticoagulation strategy are crucial for these patients to receive extracorporeal circulation surgery. This review focuses on the pathophysiology, laboratory examination, diagnosis and alternative anticoagulation strategy of extracorporeal circulation for patients with heparin-induced thrombocytopenia.