Since the 1936 seminal description of neurologic difficulties in patients with hyperglobulinemia, the "Bing-Neel Syndrome" has been applied to a range of neurologic symptoms. To clarify the central nervous system (CNS) manifestations of Waldenström macroglobulinemia (WM), we performed a literature search (years 1936 to 2008) of reports of "Bing-Neel Syndrome" or "WM affecting the CNS" (WM-CNS). We excluded cases of hyperviscosity, malignant transformation, vasculitis, and purely ophthalmologic manifestations. After analysis of symptoms, cerebrospinal fluid (CSF), imaging, and histopathology, we separate WM-CNS into (1) lymphoplasmacytoid cells infiltrating the CNS, and (2) a non-cellular form, in which other mechanisms, such as IgM deposition, might produce the neurologic symptoms.