Desmoplastic small round cell tumor presenting as an inguinal mass in a 2-year old boy

Desmoplastic small round cell tumor (DSRCT) is a highly aggressive soft tissue sarcoma that most commonly arises in the abdomen and pelvis and spreads over serosal surfaces, leading to abdominal pain or distention. Despite aggressive multimodal therapy, outcomes remain poor. It is most common in adolescents and young adults and reports in younger children are rare. We present a case of a 2-year-old male with the atypical presentation of a right groin mass, found to be DSRCT within an inguinal hernia.