Typically, an intracranial tuberculoma occurs within the brain parenchyma. Intraventricular tuberculomas are rare in the absence of systemic tuberculosis (TB), and the differential diagnosis between tuberculoma and other lesions, such as primary brain tumors, can be difficult. We report an extremely unusual case of solitary fourth-ventricular tuberculoma, which occurred in a 3-year-old female patient, with no indication of TB. This lesion appeared as a primary intraventricular tumor in the fourth ventricle in both clinical and radiological examinations. In this scenario, a surgical treatment option was pursued. Histopathological testing supported the diagnosis of tuberculoma. The patient was subsequently treated with 18 months’ therapy for tuberculous, without adverse events.