Objective To investigate the clinical and pathological characteristics, diagnosis, and differential diagnosis of clear cell papillary renal cell tumour （CCPRCT）, aiming to enhance the understanding of this tumour. Methods Clinical data, histopathological patterns, immunohistochemical and molecular assay results of four patients with CCPRCT were retrospectively analysed, followed up and reviewed in the literature. Results The study included four patients, two males and two females, aged between 39 and 73 years. One of the patients had a history of end-stage renal disease. The masses observed had a maximum diameter ranging from 1 to 3.5 cm, were well-defined, either solid or cystic, and had a color ranging from grayish-white to grayish-yellow and grayish-red. Under the light microscope, the tumour cells were arranged in tubular, papillary, vesicular or cystic shapes with varying proportions. The tumour cells were small and cubic with transparent cytoplasm. The World Health Organization/International Society of Urological Pathology （WHO/ISUP） nuclear grading was 1-2 for the tumour cells, and the characteristic nuclei were neatly arranged at the lumen edge away from the basement membrane. There was no renal sinus invasion, vascular invasion, coagulation necrosis, or other aggressive tumour features. The immunophenotypes of the tumour cells were positive for cytokeratin （CK）7, carbonic anhydrase （CA）Ⅸ, pan-cytokeratin （panCK） AE1/AE3 and vimentin, partially positive for CD10, and negative for P504S, CD117 and TFE3. The fluorescence in situ hybridization did not show any 3p deletion. Follow-up was conducted on three cases for 8-78 months, and none of them experienced tumour recurrence or metastasis. Conclusion CCPRCT is a rare renal tumour with a good clinical prognosis. It is important to differentiate it from common low-grade clear cell renal cell carcinoma and papillary renal cell carcinoma. Accurate diagnosis can improve the quality of patients' survival.