Reverse-transcriptase polymerase chain reaction (RT-PCR) analysis showed the presence of an unusual chimeric transcript, composed of an in-frame junction of exon 9 of <i>EWS</i> to exon 7 of <i>WT1</i>, confirming the histological diagnosis of DSRCT.<h4>Conclusions</h4>The present case contributes to widen the morphological spectrum of this entity; notably, the additional presence of a novel chimeric fusion transcript contributes to making the present case even more unique. This evidence concerns the gene WT1 and desmoplastic small round cell tumor.