Wilms tumor (WT) as the most frequent pediatric tumor of kidney has been shown to be associated with dysregulation of non-coding RNAs. miR-200c, miR-155-5p, miR-1180, miR-22-3p, miR-483-5p, miR-140-5p, miR-92a-3p, miR-483-3p, miR-572, miR-539 and miR-613 are among dysregulated miRNAs in this tumor. Moreover, a number of long non-coding RNAs such as CRNDE, XIST, SNHG6, MEG3, LINC00667, MEG8, DLGAP1-AS2 and SOX21-AS1 have been shown to be dysregulated in WT. Finally, distinct studies have reported down-regulation of circCDYL and up-regulation of circ0093740 and circSLC7A6 in this tumor. Dysregulation of these transcripts represents a new avenue for identification of the pathetiology of this pediatric tumor as well as design of targeted therapies.