Collecting duct carcinoma (CDC) is a rare histological type of renal cell carcinoma, which originates from the distal collecting ducts and is associated with aggressive regional and distant spread. Patients are generally diagnosed with advanced disease and the standard treatment of surgery with or without immuno/chemotherapy is not enough to effect a cure. Patients with CDC usually have a short survival time. Case reports of CDC are very rare, so there is no generally accepted therapy protocol. The clinical symptoms and radiologic findings are similar for all subtypes of renal cell carcinoma.