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Key Clinical Message Bing–Neel syndrome (BNS) is a rare manifestation in individuals suffering from Waldenström macroglobulinemia (WM). Neurological signs and symptoms in this syndrome are almost difficult to be differentiated from other common neurological manifestations of hyper‐viscosity or Waldenström‐associated polyneuropathy. In this paper, we report a new case of WM with concurrent BNS, then review the clinical picture and treatment of this syndrome.