Herein, we report the first case presentation of paraneoplastic myelofibrosis associated with cancer. Paraneoplastic syndromes occur in some patients with thoracic malignancies; however, myelofibrosis is not commonly seen in non-small cell lung cancer (NSCLC). We report a case of myelofibrosis in a patient with a new diagnosis of NSCLC that resolved after stereotactic ablative radiotherapy (SABR). In conclusion, NSCLC may evoke unexpected systemic effects that resolve with treatment.