Crow-Fukase syndrome is a disease of plasma cell dyscrasia. Congestive heart failure is the biggest complication affecting the prognosis. A 57-year-old male was admitted with edema and low grade fever. Globe and stocking type polyneuropathy, increased levels of adrenocorticotropic hormone and thyroid-stimulating hormone, serum M-protein component of the immunoglobulin A-lambda type, skin polypoid lesion, and organomegaly including cardiomegaly were observed. The diagnosis was Crow-Fukase syndrome based on these clinical features. High output heart failure and pulmonary hypertension were determined with a cardiac catheter. Diuretics and angiotensin converting enzyme inhibitor were effective to control his overhydration. The level of serum vascular endothelial growth factor was markedly increased and might be responsible for the manifestation of this syndrome with cardiac involvement.