DICER1-associated gynecological tumors include ovarian sex cord-stromal tumors (especially Sertoli-Leydig cell tumor [SLCT], but also gynandroblastoma), cervical embryonal rhabdomyosarcoma (ERMS), and ovarian sarcomas [1, 3, 35].The estimated cumulative incidence of ovarian SLCT in DICER1 syndrome patients (index and non-index case patients) is ~ 7% by the age of 60 years. The gene discussed is DICER1; the disease is Sertoli-Leydig cell tumor.