BACKGROUND: Chordomas are rare neoplasms that arise from the notochord remnant. They develop in the sacrococcygeal (50%) or cervical (15%) region and are generally regarded as a locally aggressive tumor with a slow progressive growth rate and a metastatic incidence ranging from 3 to 48%. Skin involvement by chordoma is rare, but can occur by direct extension, by local recurrence and by metastases. OBJECTIVE: To illustrate by a case report the clinical presentation and management of this disease. METHODS: We present a case of sacral chordoma with metastases over a 10-year period to the lungs, the soft tissue of the chest wall, the triceps tendon, and distant cutaneous metastases to the back and the nose. RESULTS: The cutaneous metastases were treated by excision. CONCLUSION: Chordoma is a slow growing tumor of the notochord remnant that may metastasize to the skin. Physicians and pathologists should be aware of this entity.
