Objective: The aim of the present study was to investigate the clinicopathological features of clear cell tubulopapillary renal cell carcinoma (CCTP-RCC). Methods: we reviewed the pathological slices of renal cell carcinoma cases during 2012 to 2014, and the cases diagnosed as CCTP-RCC were enrolled in our study. We observed macroscopic and microscopic characteristics of the slices, and carried out immunohistochemistry (IHC) and molecular genetics test. Results: CCTP-RCC accounted for 2.0% (4/201) in renal cell carcinoma. The average diameter of tumors was 2.0 cm (range from 1.2 to 4.5 cm). The frequencies of growth pattern of cystic, papillary, tubular and solid tumors were 100% (4/4), 100% (4/4), 100% (4/4) and 75% (3/4), respectively. A total of 4 (100%) cases were mild atypical cells. Three (75%) cases were in grade 2 and 1 (25%) case was in grade 1 in Fuhrman nuclear grade. The cells with nucleus away from the basement membrane accounted for 17.5% (range from 10% to 20%), and other locations accounted for 82.5% (from 80% to 90%). The smooth muscle was observed in all cases, and hypoplastic vascular was observed in 3 of 4 cases (75%). Of the 4 cases, 3 (75%) cases were in T1a, 1 (25%) case was in T1b. All cases were stage I tumors. CK7 was diffusely strong positive in all cases, and CD10 was negative in 3 (75%) cases, and focal weakly positive in 1 (25%) case from the IHC results. The diploid chromosome 17 was detected in 4 cases by FISH test. The mean follow-up time was 15.5 months (range from 3 to 23 months). The local relapse, lymph node and distant metastasis were not observed in all patients with CCTP-RCC. Conclusion: CCTP-RCC is a rare subtype of RCC. As for pathological diagnosis, growth pattern, cytology and interstitial performance should be analyzed synthetically. The typical immune phenotype of CCTPRCC shows that CK7 is diffuse strong positive and CD10 is negative or focal weakly positive.