The scope of this review is the report of recent findings on the developmental anatomy of the human notochord and vertebral column and the related tumors, that is chordomas. Anatomy and histology of adult intervertebral discs are also described. All the topics are completed by histochemical and immunohistochemical findings. Location and incidence of notochordal vestiges in the adult are correlated to the development of chordomas which are also described for their clinical features, skeletal distribution, spread, prognosis, and treatment. Pathology of chordomas includes morbid anatomy, histopathology, cytology, and ultrastructural findings. The seemingly distinct group of chondroid chordomas is also described. The link between chordomas and notochordal cells is stressed.