A 76-year-old male was admitted with Braunwald IIIB unstable angina and treated with intravenous heparin. Coronary angiography 20 days later revealed a severe stenosis in the left circumflex artery. During coronary angioplasty thrombus developed in the circumflex artery, extended in the left main and lead to its occlusion. Normal left coronary artery patency and flow were achieved after intracoronary and intravenous administration of abciximab, and multiple stenting. Platelet-count decrease and an ELISA assay documented the presence of heparin-induced thrombocytopenia with thrombosis syndrome (HITTS). HITTS should be suspected after acute thrombus formation during coronary angioplasty.