In twin sisters (patients 1 and 2) with young-onset vERMS we identified a region of homozygosity on chromosome 19 (7.5–13.2 Mbp) which overlaps SMARCA4. Mutations in this gene are known to be associated with Rhabdoid Tumor Predisposition Syndrome 219 (RTPS2). Here, SMARCA4 is linked to rhabdoid tumor predisposition syndrome 2.