Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a low prevalence multisystemic paraneoplastic disease. The name is an acronym composed by its most relevant clinical manifestations, which are polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. More than 95% of the POEMS syndrome cases are monoclonal for lambda light chains; however, few cases have been reported in the literature with a biclonal component. In this paper, we report a rare case of a patient who has POEMS syndrome with biclonal gammopathy. To the best of our knowledge, this is the first reported case in the literature of POEMS syndrome with expression of IgG kappa/IgG lambda biclonal gammopathy.