A 69-year-old woman had a progressive demyelinating and axonal polyneuropathy and a monoclonal gammopathy. Physical examination showed hyperpigmentation, acrocyanosis, white nails and pitting oedema. Additional tests showed polycythemia, thrombocytosis, a high serum VEGF-level, clonal plasma cells on bone marrow biopsy and sclerotic lesions of a cervical rib and the cervical and thoracal spine. POEMS-syndrome was diagnosed.