In 1971, the author reported an autopsy case of a 48-year-old Japanese man with polyneuropathy, skin hyperpigmentation, diabetes mellitus, and monoclonal gammopathy. Previously, a total of 2 cases of solitary myeloma accompanied by polyneuropathy and endocrinological disorders have been reported by Fukase et al in 1968 and by Shimomori and Kusumoto in 1970 in Japan. The author's case is the first reported non-myeloma case where polyneuropathy associated with dermatoendocrionological changes and dysglobulinemia was observed. In 1956, Crow reported the British case of a 54-year-old man with osteosclerotic myeloma, polyneuropathy, edema, skin hyperpigmentation, clubbed fingers, white nails, and lymphadenopathy. The term Crow-Fukase syndrome was first used in 1984 in a study performed by Nakanishi et al that involved 102 Japanese patients. In 1986, Bardwick et al suggested the acronym POEMS-i.e., polyneuropathy (P), organomegaly (O), endocrinopathy (E), M-protein (M), and skin changes (S)-that represents the most constant and important features of this syndrome. In this paper, the author has described 3 cases that have been reported earlier by the author and has reviewed important Japanese and international literature related to this syndrome. Recent studies have revealed that the signs and symptoms of patients with this syndrome dramatically recover on using recently developed therapies for myeloma, such as autologous peripheral blood stem cell transplantation and thalidomide therapy.