The authors report the case of chordoma with malignant cytologic featu
res, presenting as a mass lesion in the clival and infratemporal regio
n at the level of the cranio-cervical junction in an 8-year-old female
. Following gross resection of the mass, the patient subsequently deve
loped distant subcutaneous and peritoneal metastases from the lesion.
The rare histologic features, the surgical approach to the lesion, and
the follow-up management of this unique case are discussed.