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]. The gene discussed is SMARCB1; the disease is extraskeletal myxoid chondrosarcoma.