Desmoplastic small round cell tumor (DSRCT) is a rare and highly malignant tumor that mostly occurs in young males. Due to its extremely strong invasiveness and poor prognosis, the treatment of DSRCT remains a major challenge in current medical research. The comprehensive treatment strategy based on surgery, combined with chemotherapy, targeted therapy, immunotherapy has become a clinical consensus. This review summarizes the main pathogenic mechanisms of DSRCT, as well as the targets involved in treatment and their applications, including targeted therapy targets (PDGF, VEGFR, FGFR4, IGF1R, HER2, c-KIT, mTOR, AR), immunotherapy targets (PD-1, PD-L1, B7H3, GD2), and treatments related to DNA damage response. Studies have shown that treatments targeting specific targets can inhibit tumor progression and prolong patient survival to a certain extent, but the efficacy has individual differences and is still limited. Therefore, future research still needs to further explore the molecular mechanism of DSRCT and discover more accurate and effective therapeutic targets.