Objective: Cleal cell tubulopapillary renal cell carcinoma has been recently identified as a low grade renal cell tumor with distinct histological features. Method: Patient 1: 65 year old female with a history of fibromyalgia and persistent loin pain. A right renal mass, 2.8 cm in diameter, was discovered in the rheumatologist's follow up. Right nephrectomy was performed and the patient is free of disease 7 months later. Patient 2; 55 year-old female with a history of diabetes mellitus type 2, hypertension and renal failure grade 3 with a 2.5 cm in diameter renal tumor in the routine studies. She underwent tumorectomy. Results: Grossly, both were cystic tumors with gelatinous fluid and white-yellowish solid areas. Tumors showed a tubular and papillary architecture. Proliferating cells displayed clear cell cytoplasm and hyperchromatic nuclei placed in the luminal side. IHC showed positive staining with e-cadherin, EMA and CK7. Conversely, CD10, CD117 and AMACR were negative. Conclusion: Clear cell tubulopapillary renal cell carcinoma should be considered a distinct subtype of renal cell carcinoma according to its unique morphologic and inmunohistochemical features. The few cases reported so far behave in an indolent course.