Objective To explore the clinicopathologic features and treatment of desmoplastic small round cell tumor (DSRCT). Methods By summarizing the diagnosis and treatment of a DSRCT patient with liver metastasis, who was admitted to Department of Liver Surgery in West China Hospital in October 2017, and exploring its clinicopathologic features and treatment by reviewing literatures. Results This patient was generally in good condition, after the multi-disciplinary discussion between the imaging physician, the oncologist, and the liver surgeon, it was considered that there were indications of operation, and after communicating with the patient’ families, actively chose surgical treatment. The performance was successful, and this patient was treated with adjuvant chemotherapy postoperatively. The operative time for this patient was 5 hours, and blood loss was 600 mL. There was no complication occurred, such as bleeding, bile leakage, and intestinal fistula, and discharged on 8 days after surgery. This patient was followed up for 10 months, without tumor recurrence and metastasis occurred. The results of literatures showed that, DSRCT was more common in young male population, the mean age was 12–27 years old, the longest median survival time was 39.2 months, and 3-year survival rate was 20.8%–71%. Those patients who received surgery, had longer median survival time. Conclusions DSRCT is a rare aggressive soft tissue sarcoma, which is usually diagnosed with multiple organ metastases. The treatment is mainly multi-mode treatment based on surgical resection combined with radiotherapy and chemotherapy, but the overall prognosis is poor.