To our knowledge, this is the first case associated with DICER1s, consisting of MGN and both types of sex-cord stromal tumors, due to the pathogenic variant in exon 16 c.2685dupA (Phe.896IIefs*5), which, up to date, is a novel pathogenic DICER1 variant expressed with SLCT, JGCT, and MNG in our patient. The gene discussed is DICER1; the disease is sex cord-stromal tumor.