Individuals with DICER1 syndrome are at increased risk of developing: pleuropulmonary blastoma (PPB), which is the most common neoplasm associated with DICER1; among thyroid disorders, multinodular goiter (MNG) and differentiated thyroid cancers (DTC); cystic nephroma and, less commonly, Wilms’ tumor or anaplastic kidney sarcoma; ovarian tumors such as Sertoli–Leydig cell type; and embryonic rhabdomyosarcoma [2]. This evidence concerns the gene DICER1 and pleuropulmonary blastoma.