The presence of aggregates of poorly differentiated neoplastic cells with small, round to oval hyperchromatic nuclei, and scanty cytoplasm, surrounded by an abundant desmoplastic stroma, along with immunohistochemistry positivity for vimentin, desmin and NSE, and detection of the specific translocation t (11;22) (p13;q12) are the main elements for the pathological diagnosis of DSRCT. Here, DES is linked to desmoplastic small round cell tumor.