The first description of the spheno-occipital remnant of the notochord was made by Virchow,1 but he considered this jelly-like tissue to be degenerated cartilage. In describing a tumor arising from it he failed to see the relation between the tumor and the notochordal tissue. Although Muller2 had pointed out this relation in 1858, the erroneous teaching of Virchow was not corrected until the work of Ribbert3 in 1894. These authors showed conclusively that although the tissue always lies next to cartilage, it is independent of the cartilage.
In 1864 Klebs4 reported the case of a patient who died in convulsions. A tumor of this type was found at autopsy. Since that time an increasing number of cases have been reported. However, the tumor is uncommon enough to warrant the report of individual cases, particularly if the diagnosis is made at biopsy. For a review of the