A 31-year-old man was admitted to our hospital for further evaluation of heart failure symptoms. Crow-Fukase syndrome was diagnosed on the basis of findings of polyneuropathy, hepatomegaly, monoclonal hypergammaglobulinemia, and hypertrichosis. Dipyridamole-stress thallium-201 perfusion imaging, contrast left ventriculography, and coronary angiography revealed a markedly dilated and dysfunctioning left ventricle, extensive reversible ischemia with fixed defect, and multiple coronary lesions. Histopathology of myocardial biopsy specimens demonstrated ischemia-induced myocardial necrosis. These findings suggested that ischemic cardiomyopathy, probably due to inflammatory reactions of coronary arteries in Crow-Fukase syndrome, was responsible for the heart failure symptoms and left ventricular dysfunction in this patient.