The clinical manifestation, neuroimaging properties, and pathologic changes of myxoma and chordoma were studied. Their neuroimaging properties and pathologic changes of myxoma 7 cases and chordoma 13 cases were analyzed. (1) Clinical manifestation: the injury of oculomotor nerve and exorbitism is dominant in myxoma, but the injury of posterior cranial nerves is dominant in chordomas. (2) Plain skull X-ray film: local irregular punctate calcification and osseous necrosis were common in myxoma. Clivus invasion moruloid calcification and cauliflower-like calcification are always seen in chordoma. (3) CT and MRI: Location of the tumor and their relations with the adjacent structure were clear. (4) Pathologic changes: Cells are punctately arranged in myxoma, and vesicularly arranged in chordoma. The clinical study of myxoma and chordoma is not only useful for their differiental diagnosis, but can provide valuable clues for their surgical approach as well.