Erdheim-Chester disease is a rarely reported disease that can affect nearly every organ and chiefly infiltrates the connective, perivascular, and adipose tissue. The disease is a form of non-Langerhans-cell histiocytosis characterized by the proliferation of foamy histiocytes; its cardiovascular complications carry a severe prognosis. We present the case of a 29-year-old woman who was admitted for analysis of her angina. Our evaluation with use of cardiac multidetector computed tomographic angiography revealed large mediastinal soft tissue that compressed the patient's left anterior descending coronary artery. To our knowledge, this is the first report of the use of low-dose, dual-source, 256-slice multidetector computed tomography to characterize Erdheim-Chester disease that exclusively caused angina and stenosis of a coronary artery in a young adult.