Carcinoma of the papillary ducts of Bellini is a rare form of renal cancer. Patients are usually asymptomatic middle-aged men who frequently have metastatic disease at the time of diagnosis. The diagnosis is based on histological examination of the nephrectomy specimen with a precise immunohistochemical examination. Radical nephrectomy is the first-line treatment and adjuvant chemotherapy, recommended by certain authors, does not appear to improve the usually poor prognosis of these tumours.