Pulmonary hypertension is one of the well-known clinical manifestations of 
polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin 
changes (POEMS) syndrome, occurring in approximately 25-30% of the affected 
individuals. However, the histopathologic spectrum of pulmonary hypertension 
associated with POEMS syndrome has not been fully documented in the literature. 
Herein, we report an autopsy case of POEMS syndrome in a patient whose lung 
tissues showed histopathology indistinguishable from that of idiopathic 
pulmonary arterial hypertension with abundant plexiform lesions in the small 
pulmonary arteries.