The aim of this article is to provide a comprehensive review of the current state of knowledge on heparin-induced thrombocytopenia (HIT) in cardiac surgery. The management of HIT patients undergoing cardiac surgery with cardiopulmonary bypass is complex and requires an interdisciplinary and patient-tailored approach because available evidence is limited and current anticoagulation strategies have potential risks. An index case is used to discuss both the established and new perioperative therapeutic options in HIT patients undergoing urgent cardiac surgery with cardiopulmonary bypass.