Abstract: This paper reports a case of Castleman disease variant of POEMS syndrome that has no M protein but has several major and minor diagnostic criteria of POEMS syndrome including Castleman disease, vascular endothelial growth factor elevation, organomegaly, endocrinopathy, extravascular volume overload, papilledema, and skin changes. Through analyzing the clinical manifestations, laboratory examination results, and treatment responses of the patients, clinicians' understanding of atypical POEMS syndrome can be enhanced.