Chordoma is a rare malignant axial tumour that develops from embryonic remnants of the notochord. Surgery and irradiation are the standard initial treatment. However, local recurrence is frequent and cytotoxic chemotherapy is inefficient. Transient activity of imatinib, a platelet-derived growth factor receptor inhibitor, was described in a phase II study. Activity of epidermal growth factor receptor (EGFR) inhibitors (erlotinib, gefitinib) has also been shown in a few recent case reports. We describe a 68-year-old female in whom clivus chordoma recurred after surgery and radiotherapy. The tumour progressed despite imatinib treatment. A partial and sustained response (28+ months) was obtained using erlotinib, an EGFR inhibitor. Erlotinib should be evaluated in a prospective trial investigating new potential therapies against recurrent chordoma.