Background: Most histopathological variants of renal cell carcinomas (RCC) are showing distinct morphological patterns. However, there are some overlaps between some variants, which in turn, affect the prognosis. The aim of this review articleis to distinguish the recently described variants of RCCs and to illustrate the role of immunohistochemistry and genetic study in diagnosis of confusing cases. Conclusions: Applying immunohistochemical and moleculartests in addition to routine staining allow better classification of different variants of RCCs.