Chordomas are uncommon, locally invasive chordate tumors, which are mostly observed in the axial skeleton. Numerous papers have described similar patients around different anatomic locations; however, rare document previously reported that intracranial chordoma was associated with clear cell renal cell carcinoma (ccRCC). The authors report a 51-year-old male patient with a history of right radical nephrectomy for ccRCC presented to us with progressive blurred vision. Ophthalmic examination showed vision loss and visual field defects. Magnetic resonance imaging demonstrated pituitary tumor with hemorrhage, which was compressing the optic chiasm. He underwent a transnasal endoscope resection of the sellar mass. The immediate postoperative pathologic result was simply considered to be pituitary metastasis from ccRCC. After further immunohistochemical study, pathology diagnosis was made the necessary corrections to be the sellar chordoma. The authors summarize this exceptional patient and review the pertinent literature briefly.