Sarcomatoid renal cell carcinoma (SRCC) is an uncommon tumor of the renal parenchyma, representing one to five percent of all primary kidney tumors. The major symptoms are the same as in the classic renal cell carcinoma: haematuria and flank pain. The tumor consists of a bimorph feature of clear cells with areas of spindle cells and giant cells, resembling a sarcoma. Immunohistochemistry and electronmicroscopy are often necessary to prove the epithelial origin of these spindle cells. Therapy is essentially surgical but in some cases there can be a benefit of chemotherapy.