Essential thrombocythemia (ET) is a neoplastic proliferation of mature myeloid cells - in particular, megakaryocytes - leading to persistently elevated platelet count. Usual clinical presentation is related to an increase in the risk of hemorrhage and/or thrombosis. Management of ET consists of antiplatelet therapies - mainly aspirin and cytoreductive therapies. Coronary involvement in patients with ET is rare. The optimal treatment strategies for ET patients presenting with acute myocardial infarction remains unclear. Acute interventions like intracoronary thrombolytic therapy, angioplasty, and coronary-artery bypass grafting have been reported in such patients. However, several questions remain unanswered about the acute and long-term management of these patients. Herein, we report the case of a 47-year-old female who presented with acute myocardial infarction as the first clinical sign of ET, and also present the long-term follow-up of this patient.