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This article details the epidemiology and clinical manifestations of central nervous system (CNS) tuberculosis (TB), provides guidance for diagnostic imaging and CSF testing, and recommends treatment strategies for tuberculous meningitis and other forms of CNS TB, illustrating key aspects of diagnosis and management with case presentations.Although improvements in our understanding of the pathogenesis and management of CNS TB have occurred over the past 50 years, the emergence of multidrug-resistant and extensively drug-resistant TB, the advent of acquired immunodeficiency syndrome (AIDS), and the subsequent availability of highly active antiretroviral therapy that can produce the immune reconstitution inflammatory syndrome have complicated the diagnosis and treatment of CNS TB. Advances in diagnostic assays promise to increase the speed of diagnosis as well as the percentage of people with a confirmed rather than a presumptive diagnosis. Advances in precision medicine have identified polymorphisms in the LTA4H gene that influence the risk for inflammation in patients with tuberculous meningitis.CNS TB continues to be a major cause of morbidity and mortality, with the majority of people affected living in low-income and middle-income countries. Newer diagnostic assays promise to increase the speed of diagnosis and improve appropriate selection of antituberculous therapy and anti-inflammatory medications. Despite these advances, CNS TB remains difficult to diagnose, and clinicians should have a low threshold for initiating empiric therapy in patients with presumptive infection.