The DICER1 syndrome is an autosomal dominant cancer predisposition disorder that arises from pathogenic germline variation in DICER1 and is associated with a variety of benign and malignant tumors, including pleuropulmonary blastoma (PPB), cystic nephroma (CN), Sertoli‐Leydig cell tumors (SLCT), multinodular goiter (MNG), thyroid cancer, rhabdomyosarcoma, and pineoblastoma (Doros et al., 2014; Hill et al., 2009). The gene discussed is DICER1; the disease is DICER1-related tumor predisposition.