Abstract: Central nervous system (CNS) infections due to nutritionally variant streptococci (NVS) have seldom been described. The authors describe 4 patients with CNS infections due to NVS and review all published cases. Infections of the oral cavity (45%) or prior neurosurgical procedures (36%) were commonly reported before diagnosis. Common presenting symptoms included fever (64%), headache (55%), nausea and emesis (55%), and mental status changes (55%). Imaging revealed intracranial abscesses in 91% of the patients. Neurosurgical intervention was required in all patients with intracranial abscesses for diagnostic and therapeutic purposes. Isolation of NVS was made by subculture of abscess and/or cerebrospinal fluid into media cross-streaked with Staphylococcus aureus and/or supplemented with L-cysteine or pyridoxal. Penicillin or third-generation cephalosporins with or without synergistic gentamicin were the most common antimicrobial regimens used (82%). The median duration of therapy was 49 days (range, 10–118 days). Clinical outcomes were favorable for most patients.