Chordomas are rare tumors arising from the axial skeleton. The disease is characterized by slow local growth, frequent local recurrences, and rare systemic spread. Surgery and local radiation remains the mainstay of treatment with minimal role of systemic therapy. Imatinib has been shown to be active in a phase II trial with symptomatic and radiological responses. We report a case where treatment with erlotinib, an epidermal growth factor receptor tyrosine kinase inhibitor, induced symptomatic and radiological response in a patient with disease refractory to imatinib and vascular disrupting agent.