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本文报道1例原发性肾滑膜肉瘤的诊治过程。患者因腰背部酸痛不适伴全程无痛性肉眼血尿就诊，术前诊断为左肾恶性肿瘤，行腹腔镜根治性左肾切除术。术后经病理和基因检测确诊为肾滑膜肉瘤。行脂质体阿霉素化疗6个疗程+重离子放疗15 d，随访14个月未见肿瘤转移和复发。本病极罕见，确诊需依靠分子遗传学，手术和化疗是目前常用的治疗方法，多数患者预后差。“,”The diagnosis and treatment of a case of primary renal synovial sarcoma was reported. The patient was diagnosed as malignant tumor of left kidney before operation and underwent laparoscopic radical nephrectomy. Renal synovial sarcoma was confirmed by pathological examination and gene test. After 6 courses of doxorubicin chemotherapy plus heavy ion radiotherapy for 15 days, no tumor metastasis or recurrence was found during the 14 months of follow-up. The diagnosis of this disease depends on molecular genetics. Surgery and chemotherapy are commonly used at present. Most patients have a poor prognosis.