Although, most cases of adult ATRT so far are due to sporadic mutations of the SMARCB1/INI1/hSNF5 gene, Voisin et al. in his study reported a case of a familial mutation resulting in adult sellar ATRT (Voisin et al., 2019), underlining the importance of genetic testing in diagnosis of these tumors. This evidence concerns the gene SMARCB1 and atypical teratoid rhabdoid tumor.