Background Ventricular involvement in neurotuberculosis is rare. The literature regarding the characteristics of magnetic resonance imaging (MRI) in ventricular tuberculosis is very limited. Purpose To describe MRI characteristics of ventricular tuberculosis and discuss the medical treatment along with the clinical outcome. Material and Methods Ten patients (6 men, 4 women; average age, 39 years) were diagnosed with ventricular tuberculosis during a period of 3 years. Four patients had the history of pulmonary/pleural tuberculosis. The clinical and MRI features of these patients were reviewed retrospectively. Results On a brain MRI, three patients showed ependymitis associated with contrast enhancement of the ependymal lining of the ventricular walls. One patient had choroid plexitis associated with prominent swollen and marked enhancement of the choroid plexus. One patient had intraventricular tuberculoma associated with an intraventricular nodule. Two patients had both ependymitis and choroid plexitis. Three patients had both intraventricular tuberculoma and choroid plexitis. Four patients had hydrocephalus. All patients underwent intrathecal injection of isoniazid and dexamethasone combined with multidrug anti-tuberculosis treatment. All patients had a good clinical recovery, except for one who developed hemi-paralysis due to cerebral infarction. On the repeated MRI of eight patients after therapy, all lesions disappeared or decreased in size, apart from in one patient who showed ventricular separation. Conclusion MRI characteristics of ventricular tuberculosis included ependymal enhancement, swelling, and enhancement of the choroid plexus and intraventricular tuberculomas. Intrathecal injection of isoniazid and dexamethasone along with multidrug chemotherapy showed good efficacy in ventricular tuberculosis.