Chordoma is a dysontogenetic bone tumour that appears in the region of the axial skeleton. Its malignant transformation has been underestimated in the past, since the incidence of metastasis is disputed. This paper describes a chordoma of the fifth lumbar vertebra, which at first was monolocular, but in the course of the disease led to a diffuse metastasis of the spine. Specific histological findings and the differential diagnosis of the chordoma are discussed, as well as previous descriptions in the literature relating to course, diagnosis, and therapy.