A desmoplastic small round cell tumor is a malignant tumor of the abdominal and pelvic cavities. This tumor involves serosal surfaces such as the peritoneum, pleura, and scrotum. In this case report a 28-year old male presented with a desmoplastic small round cell tumor located in the abdomen along with urological symptoms. When the diagnosis was completed, the patient’s tumor had metastasized to the liver and retroperi- toneal lymph nodes. This case not only has exemplified that this tumor is rare and fatal but its prompt diagnosis is critical. However it is most likely that the diagnosis of this tumor is found when it has metastasized and this is unfortunately very late.