Although pulmonary tuberculosis is the most common form of this disease, neurotuberculosis is more severe and presents higher morbidity and mortality. Its diagnosis continues to challenge physicians all over the world. Contributing to this fact is the nonspecificity of its clinical manifestations, the low density of bacilli in the cerebrospinal fluid (CSF), and the delayed recovery of Mycobacterium tuberculosis through culture techniques. Thus, the diagnosis is largely based on suspicious symptoms, and the prognosis is directly related to the stage of the disease at the beginning of treatment. Even thought there is no consensus regarding the best therapeutic regimen, the WHO recommends using the same regimen used for pulmonary tuberculosis with a longer treatment time. It is important to note that in most cases, the doctor will not have a definite diagnosis at the beginning of the treatment. However, this should not delay the initiation of therapy. A delay in initiating treatment, in most cases, is directly associated with a poor prognosis. This review gives an overview of the current state of the neurotuberculosis research. It covers the epidemiological aspects of the infection, pathogenesis, principal clinical presentations, diagnosis highlighting neuroimaging, where a series of imaging are presented, prognosis, prevention and therapeutic regimens.