A 55-year-old Asian man first visited to our hospital with complaining of exertional dyspnea eight years ago, and was diagnosed as having idiopathic dilated cardiomyopathy. One of his siblings also suffered from idiopathic dilated cardiomyopathy. His symptoms became worse gradually, and he was hospitalized again because of disturbance of consciousness on February 21, 2001. Hemodynamic monitoring with a Swan-Ganz catheter was started, which revealed that the cardiac index was 1.1 L/ min/BSA, cardiac output 1.8 L/min, and pulmonary artery pressure 43/33 mmHg. The echocardiographic observation showed that the left ventricular ejection fraction was 32%, and serum BNP was elevated to 5,411 pg/mL. Multi-organ failure including renal and hepatic dysfunction developed because of the low cardiac output status. Continuous hemodiafiltration (CHDF) was introduced to reduce the volume overload, improve renal failure, and eliminate adverse cytokines. Although his hemodynamic status was temporarily improved after starting CHDF, weaning from CHDF was difficult and he finally died from cardiogenic shock after two month of intensive therapy. The autopsy showed thinning of the left ventricular wall, and histological examination revealed diffuse fibrous hyperplasia and myocardial fiber deficit in the ventricular myocardium. CHDF was effective in reducing the volume overload and improving renal function; however, heart transplantation is inevitable for the patients with severe heart failure due to dilated cardiomyopathy.