The spectrum of neoplasms associated with DICER1 germline pathogenic variants mostly occurs early in life and involves the lungs (pleuropulmonary blastoma), kidneys (cystic nephroma, anaplastic sarcoma of the kidney), female genitourinary system (ovarian sex cord-stromal tumors and embryonal rhabdomyosarcoma of the ovary/bladder/cervix), thyroid (nodular hyperplasia and differentiated thyroid cancer), and eye and brain (ciliary body medulloepithelioma (dictyoma), pineoblastoma and pituitary blastoma)1. Here, DICER1 is linked to neoplasm.