Although CCPRCC shares histopathogical features with clear cell RCC (CCRCC), papillary RCC and Xp11.2 translocation RCC, its immunohistochemical coexpression of cytokeratin 7 (CK7) and carbonic anhydrase 9 (CA9), and negativity for CD10, alpha-methyl-CoA racemase (AMACR), and TFE3 usually clarifies the diagnosis [3–7]. Here, CA9 is linked to renal cell carcinoma.