A 63-year-old woman with a five-month history of pulmonary epithelioid hemangioendothelioma (PEH) presented to the emergency department, due to worsening dyspnea and chest pain. The electrocardiography showed a pattern of ST-segment elevation in leads I, AVL, and poor R-wave progression consistent with anterolateral ischemia. Emergent coronary angiography revealed severe stenosis of the left main coronary artery. Then, contrast-enhanced computed tomography scan indicated the right pulmonary artery and left main coronary artery narrowing by compression of metastasized PEH. Finally, the patient died of deteriorated multi-organ failure.