Germ-line mutations in DICER1 have been described in the so-called DICER1 syndrome, a pleiotropic pediatric cancer predisposition condition causing a variety of tumor types in children and young adults, including pleuropulmonary blastoma (PPB), cystic nephroma (CN), rhabdomyosarcoma (RMS), multinodular goiter, ovarian Sertoli-Leydig cell tumor and other neoplastic conditions. Here, DICER1 is linked to DICER1-related tumor predisposition.