Isolated central nervous system (CNS) tuberculoma is rare. Central nervous system tuberculosis (TB) is associated with high morbidity and mortality despite modern methods of detection and treatment. The authors report a case of a giant cerebellar tuberculoma mimicking a malignant tumor and review the literature. A six-year-old girl, with no past medical history, vaccinated for her age, presented with a three-month history of occipitocervical cephalalgia, complicated by gait disturbances. The MRI showed a left cerebellar tumor suggestive of a medulloblastoma. At surgery, a nodular, avascular lesion was found and pathological examination confirmed tuberculoma. Intracranial tuberculoma is an uncommon variety of central nervous system tuberculosis. The prognosis is related to the rapidity of diagnosis, surgical resection and the complementary antituberculosis treatment. Intracranial tuberculoma is an uncommon variety of central nervous system infection. Prognosis is improved by a quick diagnosis, surgical removal, and associated antituberculoma therapy.