We present clinical and radiological findings in a case of collecting (Bellini) duct carcinoma (CDC). This is a rare and aggressive kidney cancer originating from the distal renal tubule. The patient underwent radical nephrectomy and the pathological report showed trabeculopapillary, partially solid adenocarcinoma infiltrating the renal capsule and sinus. Immunohistochemical, as well as mucinocarminic and PAS staining studies are necessary for diagnosis of CDC. Surprisingly, one year follow-up studies were negative for recurrent disease. Chromosomal findings are usually different from most often diagnosed clear cell renal cell cancers. Findings from the literature are briefly discussed.

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