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Non–clear cell renal cell carcinoma includes several histological variants. Tumor biology has significant effect on the duration of the disease. Prognosis at formally similar disease stages may differ despite same surgical treatment approaches to clear and non–clear cell renal cell carcinomas. Tumor sensitivity to pharmacotherapy also depends on the histological subtype. This article offers detailed discussion on current treatment options for patients with papillary, chromophobe, and other types of renal cell carcinoma.