OBJECTIVE: To investigate the diagnosis, differential diagnosis and treatment of pro-fibroproliferative small round cell tumors. METHODS: To report the clinical features, treatment and prognosis of three cases of pro-fibroproliferative small round cell tumors and to review the relevant literature. RESULTS: The pathology of our three cases (DSRCT) reported that the tumor tissue consisted of small round nests of tumor cells and surrounding sclerotic fibrous interstitium. Immunohistochemical staining showed co-expression of epithelial, mesenchymal and neurological markers. Two cases in this group were reported as abdominal tumors and lymphatic metastases, and died from 4 to 11 months of follow-up; one case was a mass in the epididymal region, which was treated with chemotherapy after transcrotal resection of the affected testis and epididymal mass, and achieved a good prognosis. CONCLUSION: Dystrophic small round cell tumor (DSRCT) is a clinically rare malignant tumor, mostly found in the abdomen, with a few invading testes, epididymis, ovaries, bones and other tissues; early metastasis of abdominal lesions, despite surgical resection and postoperative chemotherapy, still has a poor prognosis. The swelling at the testis and epididymis in the scrotum can be clearly diagnosed early and treated early with good prognosis.