Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive sarcoma that most commonly presents in males with abdominal and peritoneal invasion. There are potential curative therapeutic options, but survival remains poor. To this day, there have only been 19 reported cases of primary renal DSRCT in the literature and thoracic DSRCT has been rarely reported with very little known about effective therapy. We present a patient with a case of thoracic relapse of DSRCT after a primary renal lesion and prior history of nodular lymphocyte predominant Hodgkin Lymphoma.