Owing to age group, site, and histomorphology ES needs to be differentiated from other round cell tumors like rhabdomyosarcoma, Wilms tumor, neuroblastoma, lymphoma, desmoplastic small round cell tumor, etc. Multiple panel of immunohistochemistry markers (cytokeratin, WT1, synaptophysin, muscle-specific actin, desmin, EMA, myogenin, LCA, and CD45) is mandatory to exclude the diagnosis of other round cell tumors [10, 11]. Here, DES is linked to desmoplastic small round cell tumor.