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Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin changes (POEMS) syndrome is a rare multisystemic disorder associated with plasma cell dyscrasia, most commonly presenting with peripheral neuropathy. Due to its complex and heterogeneous clinical presentation, misdiagnosis is frequent, particularly with chronic inflammatory demyelinating polyradiculoneuropathy, which often leads to delays in appropriate management. Peripheral nerve involvement in POEMS syndrome is predominantly demyelinating, typically accompanied by early axonal degeneration. Specific clinical, neurophysiological and imaging features are key to differentiating POEMS from other acquired demyelinating neuropathies. Elevated levels of vascular endothelial growth factor (VEGF) play a central role in its pathophysiology, promoting disruption of the blood–nerve barrier and contributing to the systemic manifestations. Accurate diagnosis requires a multidisciplinary approach and is often supported by laboratory testing, including VEGF measurement and monoclonal protein screening as well as advanced imaging techniques. Therapeutic strategies are guided by disease extent and include radiotherapy for localised lesions, systemic chemotherapy and autologous haematopoietic stem cell transplantation for disseminated disease. Early identification and intervention are essential to prevent irreversible nerve damage and optimise long-term functional outcomes. Prompt recognition of characteristic findings not only enables appropriate treatment but may also significantly improve patient prognosis.