Primitive malignant rhabdoid tumor of the central nervous system are extrem
ely rare. Less than 70 cases are reported in the literature. This tumor gro
ws 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 you
ng adult. To our knowledge it is the first such case reported in the litera
ture.
We review the clinical feature, the neuradiological and therapeutical aspec
ts.