To study the clinical and pathological features and biologic behavior of multilocular cystic renal clear cell carcinoma(MLCRCCC). MethodsA case of MLCRCCC was stained with immunohistochemistry and the literature was reviewed. ResultsThe tumor was found in right kidney eighteen years before diagnosis. The majority of carcinoma cells showed cytokeratin,CEA and vimentin positivities by immunohistochemical staining. ConclusionMLCRCCC is a rare histologic subtype of renal cell carcinoma and usually cured by radical surgery. It originates from tubular epithelial cells of the kidney and appears to be a low - graded malignant neoplasm.