Collecting duct carcinoma (CDC) is an unusual variation of renal cell carcinoma. During evaluation of a 64 year old man presenting intermittent hematuria, it was detected right kidney tumor. Radical nephrectomy was performed after embolization of renal artery. He died with multiple organ métastases at the end of the first month after operation. There is no sufficient data about clinical course and treatment of the CDC. Radical nephrectomy is the treatment option for the localized disease.