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Waldenstrom′s macroglobulinemia (WM) is a rare disorder and forms 1-2% of hematological malignancies. Rarely retinal detachment precedes the diagnosis of WM. In this case report, we present 65-year-old man with retinal detachment as the inaugural manifestation in WM. Clinical manifestations in WM are related to direct tumor infiltration, circulating immunoglobulin M (IgM) and deposition into tissues, amyloidogenic properties and autoantibody activity of IgM. Diagnosis is difficult, when a rare disease manifests differently, as in our case. Delay in diagnosis can be prevented by heightened awareness of this rare disease.