The use of unfranctioned heparin and low molecular weigh heparins is very common in general medical practice and moreover in angiology and vascular surgery. Heparin-induced thrombocytopenia is a rare, yet catastrophic complication, with an incidence of 1-5% of patients receiving heparin therapy. Clinical presentation may include deep vein thrombosis, pulmonary embolism, arterial thrombosis, and skin lesions. Hemorrhage may also be present as a result of thrombocytopenia. Current diagnostic criteria include clinical scoring systems as well as laboratory tests for the identification of the specific antibodies. Efficient new alternative anticoagulant agents have been developed and are currently indicated for the treatment of such patients. The syndrome curies up to 53% thrombosis risk in untreated patients, and 28% mortality risk in patients complicated with thrombosis. This article aims in a comprehensive review of the literature for newly emerged data in the pathogenesis, diagnosis and management of heparin-induced thrombocytopenia.