Malignant fibrous histiocytoma (MFH), arising in combination with a sacral chordoma in a 70-year-old men, is described. Intermediate spindle-shaped cells demonstrating keratin positivity, showed a gradual transition between the areas of conventional chordoma, and the spindle cell areas, lending credence to the theory of a multipotential neoplasm. We chose the descriptive term "chordoma with malignant spindle cell component" in the sense that high malignant sarcomatous components exists in conjunction with chordomas in the primary tumor and the local recurrence. A review of literature is undertaken chronicling the documented associations of chordoma and sarcoma, followed by a discussion of the various causes proposed to explain this phenomenon.