Chordomas are relatively uncommon bone tumors, and fewer than 10% of cases are classified as dedifferentiated chordomas. These tumors tend to be more clinically aggressive than chordomas and have a higher incidence of early distant metastases. In this case report, we describe a 24-year-old man with dedifferentiated chordoma and multiple pulmonary metastases. Further laboratory analysis revealed an elevated serum beta-human chorionic gonadotropin level. Unfortunately, the patient's clinical condition deteriorated rapidly and he died before receiving any treatment for his cancer. However, chemotherapy may play a useful role in the management of systemic disease in patients with dedifferentiated chordoma.