Essential thrombocythemia is a clonal disorder of the myeloid stem cell that causes pathologic expansion of the megakaryocytic elements in the bone marrow, with a persistent increase in the platelet count. The disease is associated with an elevated risk of thrombosis, hemorrhage, and vasomotor symptoms. The presenting features of essential thrombocythemia can range from being asymptomatic to thrombohemorrhagic complications including acute myocardial infarction. Acute ST-segment elevation myocardial infarction due to left main trunk and ostial left anterior descending coronary artery lesions was diagnosed in a young 31-year-old man. Platelet count was markedly increased and essential thrombocythemia was also diagnosed. Because of left main disease, primary coronary intervention was not feasible and an emergent coronary artery bypass grafting was performed along with pharmacologic management of essential thrombocythemia. The early postoperative period was complicated by acute pulmonary embolism. Hydroxyurea and anagrelide were administered postoperatively, resulting in the decrease of platelet count. A succinct review of myocardial infarction in patients with essential thrombocythemia is presented, and therapeutic strategies in such patients are discussed.