A 58-year-old male presented with acute coronary syndrome (ACS) and an abnormal hematologic profile. After further testing and consultation with a haematologist, the patient was diagnosed with Polycythemia vera (PV). Coronary arteries were normal on angiography, and he was treated with dual antiplatelet regimen of Aspirin and Clopidogrel. In less than one month the patient experienced recurrent ACS with ST segment elevation. Primary percutaneous coronary intervention (PCI) was performed, and a coronary stent was implanted. However, the patient developed acute stent thrombosis as a complication. After a second PCI and replacement of Clopidogrel with Ticagrelor, no further cardiac event occurred during his hospitalization. In addition of phlebotomy, hydroxyurea was added to the medication and the patient was kept on a dual antiplatelet regimen of aspirin and ticagrelor for nine months. No cardiac event was noted over the course of a follow-up of more than one year. This is the first report describing successful treatment utilizing ticagrelor in a PV patient with repeated myocardial infarction and acute stent thrombosis.