Objective: Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive abdominal tumor typically arising in adolescent and young adult.With no large series reported, the optimal therapy modalities remain unclear.Methods: Clinical data of all consecutive DSRCT patients treated in Cancer Hospital Chinese Academy of Medical Sciences between January 2000 and December 2014 for were reviewed.Results: Ten patients with a median age of 27 years (range 14-52 years) were identified;90 % were male.9 patients underwent surgery, and the other 1 patient received biopsy.Among the 9 patients who underwent surgery, 5 patients (55.6 %) underwent complete surgical removal of all macroscopic peritoneal disease and 4 patients (44.4 %) underwent palliative resection.