Abstract
Bellini duct carcinoma is a very rare type of renal cell carcinoma (RCC), accounting for less than 1%. It arises from the distal nephron, more specifically from the collecting duct. Its morphological features are extremely variable, making its diagnosis difficult. We report the case of a 62-year old patient admitted with a painless progressive left flank swelling. CT scan showed a huge mass occupying the upper portion of the left kidney. The patient underwent radical nephrectomy. Anatomopathological examination showed collecting duct carcinoma of the kidney. Patient's evolution was exceptionally favorable: no recurrence, no locoregional metastasis and no distant metastasis.