We present a case of collecting duct carcinoma of the kidney that is an unusual variant of renal cell carcinoma, whose appearance and behavior are not well established. A 55-year-old man was admitted to our hospital with a left large renal cystic mass detected during a health examination. He had undergone radical nephrectomy under the clinical diagnosis of renal cell carcinoma. Histologically, the tumor was not typical renal cell carcinoma and immunohistochemical study was performed. The tumor cells expressed peanut agglutinin, soybean agglutinin, epithelial membrane antigen and high-molecular-weight keratin, resulting in the diagnosis of collecting duct carcinoma. He received cisplatin-based adjuvant chemotherapy in the manner of the regimen for the urothelial carcinoma, and alive with no evidence of disease for two years after surgery.