Desmoplastic small-round-cell tumor presenting as a Sister Joseph nodule

We document a rare presentation of desmoplastic small-round-cell tumor (DSRCT) in a 25-year-old male. He presented with a nodular swelling in the umbilical region that was clinically suggestive of Sister Joseph nodule. Imaging studies showed multiple nodules over the peritoneum and the gastrointestinal endoscopies showed no lesions. An excision biopsy of the umbilical lesion showed small round cells with dense desmoplasia and immunohistotyping confirmed the diagnosis of DSRCT. Desmoplastic small-round-cell tumor is notable for its poor prognosis.