Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive soft-tissue sarcoma that commonly presents late in the disease due to its nonspecific symptoms. Due to its rarity, there is still no standardized treatment regimen. A multimodal approach remains to be the cornerstone of treatment-complete cytoreductive surgery, chemotherapy, and radiotherapy. Despite advances in treatment, use of targeted and immunotherapy, an improved survival rate remains to be elusive. This paper presents a case of DSRCT in a 21-year-old Filipino patient as well as a review of the related literature of reported DSRCT cases in females and the emerging therapies for the disease.