We present a rare case of a 52-year-old woman with a chromofobe renal cell carcinoma that had sarcomatoid components. The tumor, 70x60mm in size, was incidentally found by abdominal ultrasonography. An abdominal computed tomography demonstrated a well-demarcated solid tumor arising from the upper pole of the right kidney. Histologically, the tumor was composed of two intermixed distinct morphologic components: a chromophobe renal cell carcinoma and a high-grade spindle cell sarcoma. There was a sarcomatoid area composed of highly atypical spindle cells intermingled with rhabdoid cells. A unique histological finding of this tumor was the presence of calcification and psammoma body formation throughout the tumor. We think that this case supports the existence of a tumor progression pathway from chromofob to sarcomatoid renal cell carcinoma. A literature review on sarcomatoid chromofobe renal cell carcinoma with calcification was performed