A 47-year-old man with a six-month history of progressive tetraparesis and sphincter disturbances developed tetraplegy after two years and died. A cervical MRI revealed an ill-defined, enhancing lesion from C1 to C6, internally attached to the dura mater and compressing the spinal cord. At necropsy, eight whitish nodes and diffuse thickening of the dura mater from C1 to C6 were found. Histological studies revealed multiple en plaque lymphoplasmacyte-rich meningiomas. The radiologic and pathologic aspects of lymphoplasmacyte-rich meningioma are reviewed and the atypical features of this case, such as macroscopic appearance and the histological variant, are described.