Abstract
Atypical teratoid/rhabdoid tumor (AT/RT) is a rare, fast-growing, aggressive tumor that is almost exclusively seen in the pediatric population; it has a poor prognosis despite aggressive treatment. Adult cases were thought to be exclusively of women, with a total of 23 cases reported worldwide. We herein report a case of a 35-year-old male who posed a unique clinical and diagnostic challenge. To the best of our knowledge, this is the third case of a male patient with sellar AT/RT in the world.