A 63-year-old male smoker with left chest pain was admitted for examination of an abnormal chest shadow. A chest computed tomography (CT) scan revealed a tumor in S6 of the left lung, and left pleural effusion. Histological examination by CT-guided needle biopsy revealed a proliferation of spindle-shaped tumor cells with sarcomatous features. Immunohistochemical staining confirmed the tumor cells to be of epidermal origin, indicating a diagnosis of sarcomatoid carcinoma of the lung. No distant metastases were found, and his disease was judged to be clinical stage IV (T3N2M1a). He received 5 courses of systemic chemotherapy consisting of carboplatin, paclitaxel and bevacizumab, and the tumor shrank. Systemic chemotherapy is generally ineffective for sarcomatoid carcinoma of the lung. However, in the present case this chemotherapy was effective with the addition of bevacizumab.