We describe the clinical features and post mortem findings in a patient with POEMS syndrome (P--polyneuropathy, O--organomegaly, E--endocrinopathy, M--M protein, S--skin changes), with particular emphasis on the evolution of the endocrinopathy over a three year period. In addition to gynaecomastia this case exhibited primary hypothyroidism and primary and secondary adrenal and gonadal failure. The widespread nature of the endocrine changes suggests that a circulating substance interferes with the action of trophic hormones, possibly by inhibiting second messenger production.