Primitive malignant rhabdoid tumor of the central nervous system are extremely rare. Less than 70 cases are reported in the literature. This tumor grows during the first months of life and prognosis is very poor. We report a case of primitive malignant rhabdoid tumor in a 17-year-old young adult. To our knowledge it is the first such case reported in the literature. We review the clinical feature, the neuradiological and therapeutical aspects.