The Bellini collecting duct carcinoma is a very rare form of renal cell carcinoma (1%). It presents at the stage of metastases in the very great majority of cases. The diagnosis should be considered in patients presenting with marked deterioration of the general status and/or the presence of a very large invasive renal tumour on abdominal CT scan. The overall 2-year survival rate of Bellini carcinoma is about 20%. As the prognosis is very poor, even despite radical nephrectomy, biopsy may be performed as the first-line procedure when the diagnosis is suspected. In the case of primary metastatic Bellini carcinoma, radical nephrectomy alone appears to be useless and dangerous except for analgesic purposes or in the context of new multicentre chemotherapy protocols, combining gemcitabine and cisplatin, currently under evaluation.