Pathogenic and likely pathogenic germline DICER1 variants are associated with DICER1-related tumor predisposition, a syndrome characterized by an increased risk for pleuropulmonary blastoma (PPB), pulmonary cysts, thyroid gland neoplasia (multinodular goiter, adenomas, and/or thyroid cancer), ovarian tumors (Sertoli-Leydig cell tumor, gynandroblastoma, and sarcoma), cystic nephroma, and Wilms tumor (7). The gene discussed is DICER1; the disease is Wilms tumor.