The aim of this research is to illustrate clinical and instrumental features of central nervous system tuberculosis (CNS-TB) in childhood in order to allow prompt diagnosis and adequate patient management. TB remains one of the most important communicable diseases and represents a major global health problem. Although pulmonary TB tends to be the most common form of the disease, the highest mortality and morbidity occurs with TB of the central nervous system (CNS-TB), which develops in 4% of children with tuberculosis. It has a high fatality rate and causes serious sequelae, especially during childhood. CT and MR imaging studies of 18 patients (11 female, 7 male, mean age 45.72 months) were retrospectively reviewed. A basilar enhancement was detected in all the patients. Other findings were hydrocephalus (61%), intracranial tuberculomas (17%) other nodular (11%) and ischemic lesions (11%). Edema, either perilesional or diffuse, was found in 7/18 patients. Severe ventricular dilatation was observed in 9/18 cases. Two patients with fatal outcome had severe hydrocephalus associated with extensive ischemic areas in one case and intraventricular haemorrhage in the other. Cross-sectional imaging, together with clinical data, may provide useful clues for the early diagnosis of CNS-TB and help to prevent unnecessary morbidity and mortality.