A case of clival chordoma in a 4-year-old girl is presented. The tumor regrew rapidly after it was partially removed, and the patient died after a clinical course of 11 months. An autopsy revealed a massive clival mass and widespread metastases in the dura mater, skull bone, bilateral lungs, liver, sternum, left humerus, and vertebrae. Pathological findings showed that the tumor cells were poorly differentiated, with a rare, but typical, physaliphorous appearance. The presence of epithelial differentiation proteins, mitochondria surrounded by rough endoplasmic reticulum, and desmosomes was demonstrated in the tumor cells both immunohistochemically and ultrastructurally. Thus, the tumor was diagnosed as a chordoma. The literature pertaining to intracranial chordoma in early childhood is reviewed. Rapid growth and distant metastases may occur in chordomas at a young age.