Sarcomatoid carcinoma is a rare malignancy characterized by a combination of epithelial and sarcoma or sarcoma-like components. In this study, we reported one case of pulmonary sarcomatoid carcinoma and evaluated the safety and efficacy of apatinib, a tyrosine kinase inhibitor selectively targeting vascular endothelial growth factor receptor 2, in treating this disease. The tumor mass was detected in the left lung of a 75-year-old man and showed positive immunostaining for cytokeratin (CK) 7, CK8, smooth muscle actin, CD31, and CD34. Next-generation sequencing analysis identified 4 mutations in NF1 (p.Q347Sfs*29), CDKN2A (p.G23V), ERBB3 (p.V104L), and TP53 (p.V157F) genes. The patient was given apatinib (250 mg) orally once per day. Sustained tumor regression was observed after apatinib treatment. There was no sever complication associated with apatinib therapy. In conclusion, apatinib-based targeted therapy may represent an important option for patients with sarcomatoid carcinoma.