The rare POEMS syndrome which combines hematology, bone, endocrine, neurology and skin manifestations, associated to a non-frequent osteosclerotic type of multiple myeloma is reviewed. The clinical, analytical, radiologic, immunologic, electrophysiologic and histopathologic features are detailed, as well as the different pathogenetical theories of its several manifestations, in an attempt to unify them. The different treatment schedules following the onset and corresponding prognostic are detailed.