A 70-year-old immunocompetent woman who had been radiologically diagnosed with a left parasagittal meningioma 5 years previously developed a palpable subgaleal mass and underwent neurosurgery. Histologically the tumor was composed of thickened fibrotic dural tissue, infiltrated by a follicular lymphoma, of grade IIIB. A coexistent transitional meningioma had been infiltrated by the neoplastic lymphoid tissue. Two months after surgery, the patient developed evidence of extracranial dissemination of the lymphoma to the cervical lymph nodes and was treated with polychemotherapy plus Rituximab and intrathecal methotrextate. The patient is alive and recurrence-free at 1-year follow up. A review of the literature indicates that lymphomas developing primarily in the meninges, have an indolent course and tend to be localized in areas rich in meningothelial cells.