Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive cancer that typically arises in teenage boys. As a result of its low prevalence, there have been few studies of its treatment and impact on survival. Here, we report the case of a patient who was refractory to multiple drugs but remains stable with trabectedin. To our knowledge, this is the first published report that supports antitumoral activity of trabectedin in DSRCT.