OBJECTIVE: Collecting Duct Carcinoma or Bellini Carcinoma (CDC) is a rare aggressive histological subtype. We present a case of CDC with retroperitoneal recurrence by another histological subtype of renal tumor and review of the literature.METHODS: A 59-year-old man with no relevant clinical history presented gross hematuria. At the time of diagnosis, a computed tomography (CT) showed a tumor mass occupying the left renal pelvis. Left Laparoscopic radical nephroureterectomy was performed with endoscopic intramural ipsilateral ureter disinsertion.RESULTS: The pathological diagnosis was CDC with negative surgical margins. A CT scan control was performed 10 months later, showed a left retroperitoneal tumor compatible with a local recurrence. We performed a left subcostal laparotomy with complete resection of the mass. Histological diagnosis was large cell carcinoma with components of granular cells and clear cell.CONCLUSIONS: The CDC is a rare subtype of renal cell carcinoma (RCC) and has an aggressive behavior that is associated with poor prognosis. Surgical resection remains the treatment of choice. We present the first reported case of CDC with retroperitoneal recurrence by another histological subtype of renal tumor.