Tuberculosis of the central nervous system is the most severe form of Mycobacterium tuberculosis infection, which accounts for approximately 1% of the cases of active tuberculosis. Most frequently, it manifests as tuberculous meningitis (TBM). Despite treatment, TBM is associated with high rates of neurological sequelae and mortality. Immunocompromised individuals such as those infected with human immunodeficiency virus are at higher risk for developing TBM. The clinical presentation of the disease is nonspecific and the diagnosis can be difficult. If TBM is suspected, empirical treatment must be initiated immediately, even before the diagnosis is confirmed. Antimicrobial treatment guidelines are based on those used to treat pulmonary tuberculosis. In the case of drug-resistant TBM, regimen changes are needed. Adjunctive glucocorticoid therapy has shown a positive impact on reducing mortality. In addition, supportive treatment is an integral part of the management of TBM.