Bing-Neel syndrome is a rare neurologic complication of Waldenström’s macroglobulinemia, characterized by infiltration of the central nervous system by clonal lymphoplasmacytes. We present a rare clinical case of a patient, who one year after the diagnosis of Waldenström’s macroglobulinemia, progressed with diverse neurologic presentation and cerebrospinal fluid involvement. The diagnosis was based on magnetic resonance imaging and flow cytometry detection of clonal B-cells in the cerebrospinal fluid. Bing-Neel syndrome should be considered in patients with neurologic symptoms and a history of Waldenström’s macroglobulinemia.

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