We present a rare clinical case of renal cell carcinoma from the collection tubules-carcinoma of Bellini. A 69 years old patient, enters in our department with symptoms of massive hematuria. From the studies carried out-blood tests, CT and cystoscopy there are no data of neo process of lower urinary tract, but shows horseshoe-shaped kidney, cystic deformation of the left kidney and aortic dissection. A left radical nephroureterectomy was performed, with preoperative diagnosis of renal tumor at stage T3. The histopathological diagnosis was Bellini duct carcinoma of papillary tubular type. The patient currently remains disease -free.