Crow-Fukase syndrome or POEMS syndrome is a variant of plasma cell dyscrasia that is characterized by polyneuropathy, organomegaly, endocrinopathy, the presence of M-protein in serum, and dermatological changes. A 60-year-old man presented with features of Crow-Fukase syndrome, such as the presence of M-protein in serum, dermatological changes, and osteosclerotic changes, but did not have polyneuropathy. To our knowledge, this is the first case of Crow-Fukase syndrome in which the presence of hypertrophic cardiomyopathy has been confirmed by a left endomyocardial biopsy. The findings suggest that hypertrophic cardiomyopathy may be a manifestation of organomegaly in patients with Crow-Fukase syndrome.