Collecting duct carcinoma(CDC) of kidney is a highly aggressive tumor with an extremely poor prognosis. This is a renal cell carcinoma that comes from the distal segment of Bellini's collecting tubes in the kidney and makes less than 2% of the total kidney mass. CDC is included as a specific entity in the classification of kidney tumors. Most commonly affects men (middle age 55 years). Tumors are predominantly symptomatic and some of the symptoms that are characteristic of other kidney tumors such as hematuria, pain, renal mass appear. Even 1/3 of patients already have developed metastases during diagnosis. Typical Bellini tumors are macroscopic white to gray and have a firm consistency on the presentation. Microscopic characteristics of the CDC allow proper classification of this tumor.Tumor cells show a typical high degree of atypia, frequent pathological mitosis. Immunohistochemical expression of CDC shows the origin of tumor from the collecting tube of distal nephron. The treatment that provides the longest survival rate is a surgical procedure if performed in patients with small tumors. Also except going on operation patients can be treated with other methods such as chemotherapy, radiotherapy and immunotherapy. Survival is very low, and usually is not longer than two years.