SMARCB1 mutations have been implicated in the pathogenesis of several malignancies, including atypical teratoid rhabdoid tumor, malignant rhabdoid tumor, epithelioid sarcoma, renal medullary carcinoma, myoepithelial carcinoma, epithelioid malignant peripheral nerve sheath tumor, and extraskeletal myxoid chondrosarcoma [8]. Here, SMARCB1 is linked to atypical teratoid rhabdoid tumor.