Hyperviscosity syndrome (HVS) is a life-threatening complication. The clinical manifestations include a variety of symptoms like visual symptoms, altered mental status, stroke or congestive heart failure. Prompt treatment is needed to avoid progression to multisystem organ failure. We report a case of a 73-year-old man with a 3-months history of headaches and altered mental status. His neurological exam showed symmetrical decreased pain, thermal and tactile sensitivity in the upper and lower limbs and symmetrical decreased muscle strength in the lower limbs. His eye exam showed retinal hemorrhages and dilated retinal veins. His blood counts showed anemia, increased C-reactive protein, sedimentation rate and serum viscosity. He had an elevated immunoglobulin M and serum immunofixation revealed Ig M-kappa paraprotein. The bone marrow aspiration and biopsy revealed a Waldenström Macroglobulinemia. He was diagnosed with HVS and was treated with plasmapheresis, chemotherapy and fluids.HVS diagnosis requires a high degree of clinical suspicion, andclinicians should be aware of suggestive clinical and laboratory findings.