•Embryonal rhabdomyosarcoma of the uterine cervix and ovarian Sertoli-Leydig cell tumors are associated with DICER1 mutation•DICER1-associated tumors should prompt genetic counseling and testing•Somatic and germline genetic mutation profiles can be used to differentiate second primary from recurrent tumors.
Keywords: DICER1 syndrome; Embryonal rhabdomyosarcoma; Sertoli-Leydig cell tumor.