Chordomas are rare malignant tumors that rise from notochordal remnants of the developing spine. Distant metastases are rare and mostly occur in patients with local recurrence. In the case reported, 14 years after radical resection of a sacral chordoma, a distant metastasis to the left pulmonary vein with large intracardiac mass, location never described before, was diagnosed. No evidence of local recurrence was observed. Although the intracardiac tumor part was successfully resected, the patient died of heart failure.