Objective To improve the diagnosis and treatment of renal mucinous spindle cell carcinoma, and the knowledge of the type of kidney cancer. Methods The clinical date of 1 case of renal MTSCCa was reported.The case of patient was a 38–year–old women with abdominal discomfort and hematuresis.Ultrasound tip: the upper pole of the right kidney mixed echo placeholder, tumor and the surrounding is obvious blood flow signal.CT revealed a low density shadow in the upper pole of the right kidney, involving the right renal vein, partial filling defect, visible in the inferior vena cava are visible low density shadow, uneven enhancement after the reinforcement, and has delayed reinforcement.MRI shows: the upper pole of the right kidney huge soft tissue mass, T1WI shows low signal, T2WI show slightly higher signal, uneven enhancement and have delayed reinforcement.Preoperative diagnosis: right renal clear cell carcinoma with right renal vein and inferior vena cava tumor emboli.With kidney cancer radical resection and take out inferior vena cava tumor emboli, in the operation right kidney tumor, about 10 cm×20 cm, right renal vein and inferior vena cava tumor emboli in formation, inferior vena cava tumor emboli within about 8 cm, the clinical and pathologic feature was analyzed and discussed with literature review. Results The operation smoothly.Pathological findings suggested higher grade mucinous tubular and spindle cell carcinoma; microscopically a large number of ingredients mixed bundle arrangement of spindle cells, nuclear fission as easy to see.Immunohistochemical staining: Vimentin±, Syn+ , NSE+ , CD99+ , Ki–67+ 70%.According to the 2010 AJCC for kidney cancer TNM staging, the pathological pT3cN0M0.According to the Robson stage Ⅲ.The patient reserved interferonα–2a associate with interleukin–2 theraph after a month.During treatment in patients with fever and other flu symptoms, weekly review of routine blood, liver and kidney function has no obvious abnormalities.The patient died of pulmonary embolism, liver, lung multiple metastases two months later. Conclusions Renal mucinous tubular and spindle cell carcinoma is a rare cell types in the kidney pathological classification, women in high incidence, higher pathological types, worse prognosis. Key words: Mucinous tubular and spindle cell carcinoma; Tumor of kidney; Prognosis