Listeria monocytogenes infection of the central nervous system is often not recognized and treated appropriately in the crucial early stages of the disease. Most consider patients with underlying disease or immunocompromised states to be at risk, although healthy individuals may present with a neurologic syndrome caused by L. monocytogenes. Earlier suspicion and treatment remains our best means of reducing the morbidity and high mortality rate of this treatable disease. In addition to meningitis and meningoencephalitis, infection of the brainstem (rhomboencephalitis) is challenging to recognize and therefore initiate appropriate early therapy. Cerebritis and abscess can also occur. Furthermore, empirical therapy for meningitis or the other manifestations of nervous system involvement is often inadequate. This review addresses the clinical microbiology, pathogenesis, spectrum of neurological involvement, and treatment of central nervous system infection related to L. monocytogenes.