Germline mutations in DICER1 cause the DICER1 syndrome, a multi-tumor condition in which the afflicted family member exhibits an increased risk of pleuropulmonary blastoma, cystic nephroma, ovarian sex cord-stromal tumors (most commonly Sertoli-Leydig cell tumors), various sarcomas as well as multinodular goiter [7–9]. Here, DICER1 is linked to multinodular goiter.