Some types of pediatric ovarian neoplasms have been associated with specific cancer predisposition syndromes, including Peutz–Jeghers syndrome, hereditary leiomyoma renal cell carcinoma syndrome, DICER1, rhabdoid tumor predisposition syndromes, nevoid basal cell carcinoma syndrome, and von Hippel–Lindau syndrome (26, 27). Here, DICER1 is linked to nevoid basal cell carcinoma syndrome.