In an attempt to contribute data to the ongoing discussion on DICER1 alterations in ERMS, we report the clinicopathological characteristics of a cohort of genitourinary ERMS including DICER1-mut and DICER1-wt tumors by applying targeted DNA sequencing and performing comparative, genome-wide DNA methylation and copy number variation (CNV) analyses. Here, DICER1 is linked to embryonal rhabdomyosarcoma.