Neurofibrosarcomas are rare tumors usually arising in somatic soft tissues or peripheral nerves. Four cases of metastatic neurofibrosarcoma to the spine have been reported before. The current case is unusual because of the presence of two distinct, metachronous spinal metastasis and lung metastasis. A 30-year-old woman with neurofibromatosis and a history of previous neurofibrosarcoma resection presented with back pain. Radiologic evaluation revealed a lytic lesion of the eleventh thoracic vertebra. A transthoracal corpectomy, reconstruction by Harms' cage and posterior instrumentation, and fusion were carried out. After the completion of adjuvant chemotherapy, a solitary pulmonary nodule was detected. Shortly after resection of the metastatic pulmonary nodule, the patient complained of acute onset low-back pain. Radiologic assessment revealed another lytic lesion in the L5 vertebra after 6 months. Again, a corpectomy, anterior and posterior instrumentation, and fusion were carried out. Eight months after the second spinal resection, another solitary pulmonary metastasis was diagnosed and resected. The patient's health status suddenly deteriorated 26 months after the initial spinal metastatectomy, and she died. Though local control can be achieved in more than 80% of the patients with neurofibrosarcoma by wide surgical resection followed by adjuvant chemo- and radiotherapy, most patients die of systemic metastasis. The current patient survived 50 months after the initial resection of a forearm neurofibrosarcoma. Despite achieving local control, she died due to systemic recurrence. Prolonged survival with the help of chemo- and radiotherapy justifies our aggressive surgical strategy for the treatment of spinal metastasis in order to achieve neurologic cure and spinal stability.