A young woman was referred to our institution for the appearance of a single pulmonary nodule at a routine follow-up computed tomographic scan of the thorax. She had been operated on 4 years earlier for a parachordoma of the iliopsoas muscle. Wedge resections were accessed through a mini-thoracotomy, and the pathologic examination confirmed the diagnosis of parachordoma lung metastases. Adjuvant chemotherapy (Glivec, 400 mg/daily [imatinib; Novartis, Basel, Switzerland]) was indicated and was administered for 10 months. At the time this case report was written, the patient was alive with no sign of tumor recurrence. We believe that metastatic parachordoma to the lung has been previously reported only once in the English literature. Our case adds evidence that parachordoma, despite its generally indolent behavior, can metastasize to the lung.