Although the present case showed typical histological features and immunohistochemical profiles, successful detection of the EWSR1-WT1 gene rearrangement involving t(11,22)(q13;q12) by FISH and RT-PCR assays using FFPE tissues conclusively confirmed the diagnosis of DSRCT in this uncommon location. The gene discussed is EWSR1; the disease is desmoplastic small round cell tumor.