Objective: To discussed the clinical and histopathologic features, differential diagnosis, imaging features and prognosis of pulmonary pleomorphic carcinoma (PPC). Methods: Histopathology and immunohistochemistry were used for the retrospective analysis of 10 patients with PPC, who underwent surgical resections in Wuxi People’s Hospital from March 2012 to April 2018. Results: All patients were male, with varying ages from 59 to 71 years. Eight patients had previous heavy smoking history, and the remaining two did not smoke or drink. Seven patients occurred with initial symptoms including cough or expectoration, and three of them were with haemoptysis. In this group, the average diameter of the tumor was 6.2 cm, and the maximum and minimum tumor diameter were 9.5 cm and 2.8 cm, respectively. Among these ten patients, seven had PPC in the right lung and three had PPC in the left lung. Malignant epithelial components and polymorphic components were found in these patients. Specifically, malignant epithelial components include 9 cases of adenocarcinoma and 1 case of squamous cell carcinoma, and polymorphic components include malignant spindle cells and/or giant cell components. Epithelial components differentially expressed CK, AE1/AE3, CK7, TTF-1, Napsina, TTF-1, CK5/6, Vimentin and so on, with varied value-added Ki-67 index (40%–80%). Additionally, four patients occurred with lymph node metastasis. After the surgery, nine patients obtained postoperative follow-up with the shortest survival period of 7 months after surgery. The longest survivor is still alive at the 41th month after the surgery. Conclusion: PPC is a rare and highly malignant tumor, which occurs mostly in middle-aged smoking male. Most PPC can be differentially diagnosed by histopathology and immunohistochemistry. Currently, surgery is a preferred treatment for PPC, but prognosis of PPC is poor.