OBJECTIVE: To present our experience with Bellini duct carcinoma, a rare form of renal adenocarcinoma with well-defined histological, cytogenetic and immunohistochemical characteristics. The literature is reviewed. METHODS/RESULTS: We reviewed the records of 430 patients with renal tumor that had been treated over a 10-year period. Only 6 cases with Bellini duct carcinoma were found. The mean age was 60 years, all patients were male and hematuria and lumbar pain were the most common clinical manifestations. Diagnosis was made by imaging techniques (US, urography, CT). The definitive diagnosis was based on the histological and immunohistochemical findings following nephrectomy. The one-year and three-year survival rates were 50% and 0%, respectively, indicating the aggressive nature of this variant of renal adenocarcinoma. CONCLUSIONS: Bellini duct carcinoma is an uncommon variant of renal carcinoma with nonspecific clinical features. It is a very aggressive tumor type whose treatment is only by nephrectomy and the outcome is poor.