Sarcomatous transformation in chordoma (dedifferentiated chordoma) is a very rare condition and has been emphasized as a distinct entity because of its more aggressive clinical course. Here we describe a case of dedifferentiated chordoma (chordoma associated with malignant fibrous histiocytoma) arising from the sacrococcygeal region of a 55-year-old woman. The results of immunohistochemical stain in the chordoma area were strong positive for cytokeratin, epithelial membrane antigen and S-100 protein. The spindle and giant cells in the transitional areas of chordoma and malignant fibrous histiocytoma were positive for cytokeratin and epithelial membrane antigen in addition to vimentin and alpha-1-antichymotrypsin. The spindle and giant cells in the central area of malignant fibrous histiocytoma were negative for cytokeratin and epithelial membrane antigen, but positive for vimentin and alpha-1-antichymotrypsin. This supports the pathogenesis of sarcomatous transformation from chordoma.