Heparin-induced thrombocytopenia (HIT) is an immune-mediated adverse drug reaction to heparin products leading to a prothrombotic state. Devastating clinical sequelae may result, including venous or arterial thromboembolism, limb amputation, and death. Heparin cessation alone is insufficient to manage HIT. Pharmacotherapy with argatroban or lepirudin is essential. This article reviews the pathogenesis, diagnosis, and pharmacotherapy of HIT.