To date, Dicer1 syndrome has been associated with pleuropulmonary blastomas, cystic nephroma, rhabdomyosarcoma, multinodular goiter, thyroid cancer, Sertoli–Leydig cell tumor, ovarian sex cord-stromal tumor, ciliary body medulloepithelioma, pituitary blastoma, pineoblastoma and other neoplasms [37,42,43,44,45]. The gene discussed is DICER1; the disease is Down syndrome.