Abstract: Objective To investigate polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome manifested by Castleman's disease, in order to improve the understanding of the diagnosis and treatment of this disease. Methods One Castleman's disease patient with POEMS syndrome who were admitted to Department of Hematology, the Affiliated Hospital of Xuzhou Medical University was selected to analyze clinical data. Furthermore, relevant literature at home and abroad was reviewed. Results The patient was admitted to Department of Hematology, the Affiliated Hospital of Xuzhou Medical University due to "swollen lymph nodes for half a year" on June 2015. Through aspiration biopsy of cervical lymph nodes, the patient was pathologically diagnosed with Castleman's disease (plasma cell type). PET/CT results were consistent with the manifestation of Castleman's disease, while lymphoma was not excluded. The serum M-protein was determined in another hospital and the results indicated that IgA λ-type M-protein positive serum free light chain (sFLC)-λ was 40.1 mg/L, sFLC-κ was 101.0 mg/L; soluble protein electrophoresis (SPE):M-protein% 4.5%, β-2 microglobulin 10.1%, albumin 51.7%; Ig3 item:IgA 10.67 g/L. According to previous lymph node biopsy results and related clinical manifestations, the patient was diagnosed with POEMS syndrome. On November 2018, autologous stem cell transplantation was performed. After transplantation, the patient was regularly followed-up and re-examined for more than three years. Although some enlarged lymph nodes could not be relieved, there were no symptoms related to POEMS syndrome. No special abnormality was found in physical examination and laboratory results. Conclusions Both Castleman's disease and POEMS syndrome are rare diseases that are easily misdiagnosed and missed in clinical practice. The cases of Castleman's disease and POEMS syndrome at the same time are very rare. The association, pathogenesis and prognostic factors of Castleman's disease and POEMS syndrome are still unknown and need to be investigated in more depth.