POEMS syndrome is a potentially fatal disease, and prediction of prognostic factors for POEMS syndrome is important for clinicians. Papilloedema is an early sign of the syndrome. The present study was carried out to evaluate whether papilloedema is a prognostic factor for POEMS syndrome. Between January 2003 and December 2012, 94 patients with POEMS syndrome were enrolled in this study. The patients were divided into groups with and without papilloedema. Logistic regression was performed to identify risk factors related to papilloedema. Prognostic factors were analysed with Cox proportional hazard regressions for POEMS syndrome-related prognoses, and survival curves were plotted using the Kaplan-Meier method and compared by the log-rank test. Papilloedema was found in 52.1 % of the patients with POEMS syndrome. Raised intracranial pressure [hazard ratio (HR) 3.06, 95 % confidence interval (CI) 1.24-7.41; P = 0.011] and elevated cerebrospinal fluid protein levels (HR 2.03, 95 % CI 1.07-4.51; P = 0.043) were independently associated with papilloedema. Papilloedema, decreased diffusing capacity of the lung for carbon monoxide (DLCO) and treatment with corticosteroids alone were related to poor prognosis in POEMS syndrome. In multivariate analysis, papilloedema (HR 1.58, 95 % CI 1.05-2.46; p = 0.027) and decreased DLCO (HR 2.17, 95 % CI 1.12-3.39; p = 0.023) were independent factors for POEMS syndrome-related death. Papilloedema and decreased DLCO are important prognostic factors for patients with POEMS syndrome, which can help clinicians predict the risk of mortality and provide better medical care for these patients.