To report one case of collecting duct (Bellini) carcinoma and to review the national bibliography. To analyze the clinical, diagnostic, and therapeutic features of this malignant renal tumor.A 36-year-old male presenting with monosymptomatic hematuria and clot retention was diagnosed of left renal mass suggestive of renal carcinoma. Radical nephrectomy with interaortocaval lymphadenectomy through an "L" abdominal incision (inverted Makuuchi) and postoperative radiotherapy were carried out. The pathologic report showed a collecting duct (Bellini) carcinoma. The patient died 34 months after surgery, having been treated with two courses of chemotherapy and palliative vertebral radiotherapy.The collecting duct (Bellini) carcinoma is an infrequent malignant renal tumor. It does not differ from the clear cell adenocarcinoma either clinically or epidemiologically. However, it presents its own embryological, pathologic, immunohistochemical, and cytogenetic characteristics. Radical nephrectomy is the treatment of choice. Prognosis is ominous because of the aggressive behaviour and diagnosis in advanced stages.