The prognosis is poor for patients with advanced pleomorphic carcinoma of the lung due to the generally limited response to chemotherapy and/or radiotherapy. It has been suggested the production of granulocyte colony-stimulating factor (G-CSF) by cancer cells may aggravate the disease progression. We herein report a case of a 73-year-old Japanese man with advanced G-CSF-producing pleomorphic carcinoma of the lung. First-line chemotherapy with carboplatin and paclitaxel had been suspended. Subsequent radiotherapy achieved a moderate volume reduction and an amelioration of the excessive G-CSF-related complications. Six cycles of second-line chemotherapy with docetaxel administered with good results. These combined treatments resulted in long term survival without progression of the disease.