Abstract: Desmoplastic small round cell tumor (DSRCT) is a rare malignant tumor, which is prone to occur in teenagersDesmoplastic small round cell tumor (DSRCT) is a rare malignant tumor that often occurs in adolescents. Early diagnosis is difficult and the prognosis is poor. In this case report, the ultrasonography of DSRCT showed that the huge solid mass was in the abdomen with unclear boundary, irregular shape, insufficient blood supply but without obvious liquefaction and calcification. The masses encircled the vessels, but no evidence of vascular invasion. Intrahepatic metastases with peripheral hypoechoic aureole and abdominal lymph node metastases were observed. The tumor mass compressed adjacent tissues and organs, causing bilateral hydronephrosis and bone erosion. In a word, the ultrasonographic characteristics could be used for diagnosing the DSRCT in the clinic.