A 51-year-old man visited our hospital with a chief complaint of left renal mass detected by ultrasonography in another hospital. Enhanced computed tomography (CT) revealed a hypovascular tumor measuring about 9 cm in the left kidney, tumor thrombus in the inferior vena cava and paraaortic lymph nodes metastases. Radical left transperitoneal nephrectomy with removal of the thrombus from the left renal vein were performed. Paraaortic lymph nodes dissection was partially performed because of the solid adhesion to aorta. Histological examination revealed collecting duct carcinoma (CDC). He was treated with systemic chemotherapy (gemcitabine plus nedaplatin) followed by sunitinib. Duration of stable disease after initiation of therapy was seventeen months, and survival after surgery was 31 months. It would be worthwhile to prospectively evaluate the antitumor activity of tyrosine kinase inhibitors against metastatic CDC.

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