Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive soft tissue sarcoma. It predominantly occurs in young adults, with a mean age of 25 years, and exhibits a notable male predominance. DSRCT most frequently arises in the peritoneal cavity; however, involvement of the bones, lungs, liver, and, very rarely, the pleura has also been reported, with fewer than two dozen pleural cases described in the literature. The most common clinical manifestations include chest pain and dyspnea. Back and neck pain, attributed to invasion of adjacent structures, may also occur, along with systemic features such as weight loss. Pleural disease is typically unilateral. DSRCT is defined by the chromosomal translocation t(11;22)(p13;q12), which generates the pathognomonic EWSR1-WT1 fusion gene, resulting in the upregulation of multiple oncogenic pathways. Diagnosis relies on cytological or histological evaluation, with confirmation of the translocation using fluorescence in situ hybridization (FISH). In cases where cytology is inconclusive, larger tissue samples may be required for diagnosis, which can be obtained through core needle biopsy or video-assisted thoracoscopic surgery (VATS). Therapeutic principles for pleural DSRCT are extrapolated from management strategies used in peritoneal DSRCT, with additional adaptations drawn from the treatment of pleural malignancies such as mesothelioma. Standard therapy consists of neoadjuvant chemotherapy, followed by cytoreductive surgery and consolidative radiotherapy or chemotherapy. The prognosis remains poor, paralleling that of peritoneal DSRCT.