Fourteen years after its initial discovery in S. cerevisiae, SMARCB1 was characterized in humans as a bona fide tumor suppressor gene biallelically inactivated in particularly rare and lethal early childhood cancers known as malignant rhabdoid tumor of the kidney (MRT) and atypical teratoid rhabdoid tumor (ATRT) [4]. The gene discussed is SMARCB1; the disease is neoplasm.