WHO officially identified clear cell papillary renal cell carcinoma（CCPRCC）as a new type of renal cell carcinoma in 2016. This tumor type can be encountered in a sporadic setting or can occur in end-stage renal disease or even VHL syndrome，with relatively characteristic histopathological morphology，immunophenotype and molecular genetics. It needs to be identified with clear cell renal cell carcinoma，papillary renal cell carcinoma or renal carcinomas associated with Xp11.2 translocations. In view of the biological behavior of CCPRCC is relatively inert or low-grade malignant，therefore accurate diagnosis plays an important role in clinical treatment and judging prognosis.