Abstract: Objective To improve the diagnosis and treatment of retroperitoneal liposarcoma.Methods We reported one case of huge retroperitoneal liposarcoma in our hospital.The clinical characteristics,diagnosis and treatment were discussed,and the literatures were reviewed.Results The patient received the left retroperitoneal tumor resection and partial resection of the left kidney after the imaging diagnosis.Postoperative pathological results indicated retroperitoneal liposarcoma.No tumor recurrence and metastasis ocurred in the follow-up of 20 months.Conclusion Retroperitoneal liposarcoma is rare,with no specific clinical manifestations.The application of CT is helpful to the diagnosis of this rare disease.Surgery is the preferred method for the treatment.