Desmoplastic small round cell tumor (DSRCT) is a relatively uncommon and highly aggressive malignancy in young males. It is associated with a poor outcome, due in part to missed diagnosis. To characterize the clinical pathological features of DSRCT in Chinese patients and to find out the characteristics of treatment and prognostic factors, the authors collected and analyzed the clinical information of 48 cases. A total of 48 cases of DSRCT between March 1995 and March 2012 were retrospectively reviewed and analyzed. The clinical information, histological results and survival data of the patients were collected. Median age was 26.96 ± 14.09 years with a range of 6-66 years. Thirty-three patients (68.75%) were seen before 30 years old, and 15 patients (31.25%) were diagnosed after 30 years old. The male-to-female ratio is 3.36 :1. Among them, 37 cases presented with tumors in the abdominal or pelvic cavity; the other 11 cases had extra-abdominal tumors. The most common symptoms were abdominal pain (19/48, 39.58%) and palpable mass (12/48, 25.00%). The percentage of patients received surgery, complete surgery, and chemotherapy was 79.17%, 37.50%, and 52.08%, respectively. Median follow-up duration was 2.67 years. Median overall survival for all patients was 24.33 months (95% CI: 9.74-38.92 months) and median event-free survival for all patients was 8.00 months (95% CI: 5.13-10.89 months). Univariate analysis revealed that surgery, effective debulking surgery, chemotherapy and any two or more combined therapeutics were significant prognostic factors for longer overall survival (p < 0.05). Cox regression analysis showed complete surgery was an independent prognostic factor. Standard therapy for DSRCT consists of combination of surgical resection and postoperative chemotherapy. Complete surgery is an independent prognostic factor and should be further investigated.