Chordomas are rare tumors derived from notochord remnants occurring primarily in the sacrum, clivus, and cervical regions. Exceptionally, these tumors occur in children, though usually in the sacrum. Eight cases of clivus chordoma have been described in preteenagers. In this report, a clival chordoma with unusual radiologic features is described in an 11-year-old boy. The literature regarding this entity is reviewed.