Abstract: Clear cell sarcoma (CCS) is a rare, aggressive soft tissue sarcoma that primarily affects adolescents and young adults and typically involves tendons and aponeuroses.Currently, systematic treatment based on surgery is mainly adopted for CCS; the outcomes of chemotherapy and radiotherapy are poor due to the high resistance.With the progression in research regarding CCS pathogenesis, targeted therapy and immunotherapy are becoming increasingly important in the treatment of CCS.However, CCS has a high rate of local recurrence and distant metastasis, so it is urgent to identify comprehensive treatment regimens.This article reviews the current status and progress of treatment for CCS to provide information for clinical practice.