Abstract: As part of the workup of polyneuropathy, neurologists often encounter patients with paraproteinemias, also called monoclonal gammopathies. Given the relatively rising prevalence of both polyneuropathy and paraproteinemias in our aging population, this coexistence is frequently due to chance. Still, certain types of paraproteinemias have a well-defined causal role in the development of polyneuropathy, and therefore has implications in their subsequent work-up and management. Once a monoclonal gammopathy has been deemed as “benign” or of undetermined significance, looking at the heavy chain subtype is paramount in deciding whether it is related to a polyneuropathy. Conversely, polyneuropathies associated with monoclonal gammopathies tend to have a distinct clinical, serological, and/or electrodiagnostic phenotype that helps in establishing an association. Recognizing this association will determine appropriate workup and management.