Dedifferentiated chordoma is a rare primary malignant bone cancer. Most cases of dedifferentiated chordoma (DC) are transformed from recurrent chordoma after surgical resection or radiation. The prognosis of DC is extremely poor because of the aggressive nature of the tumor and the potential distant metastases. We report a case of de novo DC of the sacrum in a patient without prior surgical procedure or radiation treatment. A complete review of reported cases sourced from reports published in English literature is discussed and expanded upon and conclusions on the treatment of DC are presented.