Chordomas are extremely rare among the spinal tumors in pediatric age. The sacrococcygeal region is the typical site of such a lesion. A case of spinal chordoma in a child 4 years old, at the T12-L1 level, is reported. The tumor was surgically removed and a local radiotherapy was performed, but 9 months later spreading of lung metastases was observed. The unusual site along the vertebral column and the metastatic potential of this tumor are discussed. The very few cases reported in the literature are analyzed.