Rhabdoid tumor (RT) is an uncommon childhood neoplasm that typically arises within the kidney. It is characterized by an aggressive clinical course. Since its description in 1978, several cases of primary extrarenal RT, including a CNS localization, have been reported. The first case in the CNS was reported in 1985 and was defined as “rhabdoid tumor” initially, and was classified as grade IV in the most recent classification of the World Health Organization under the term of “atypical teratoid/rhabdoid tumor”. Nearly 200 cases of atypical teratoid/rhabdoid tumor of the CNS have been reported to date, most of them occurring in childhood. We report a case of primary RT of the brain located in the right frontal lobe with the clinical, radiographic and pathological features presenting at an unusual age. This tumor, which was composed purely of rhabdoid cells with no additional primitive neuro-ectodermal, epithelial and mesenchymal components, was in a 27-year-old male patient. In conclusion, RT should be considered also in the differential diagnosis of intracerebral neoplasms of adult patients.