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Pulmonary spindle cell carcinoma (PSCC) is a highly malignant carcinoma that often exhibits the histopathological characteristic of cell pleomorphism.Here, we report a PSCC case in a 59-year-old woman with multiple metastases. The pathological manifestation of this case did not show high-grade pleomorphism or atypia, and was easily mimicked by other borderline or low-grade malignant tumors such as an inflammatory myofibroblastic tumor (IMT).Based on the results of immunohistochemistry and fluorescence in situ hybridization after the operation, IMT was ruled out, and PSCC was confirmed.Resection of the right middle lobe and dissection of the lymph nodes were performed. The patient was given 2 cycles of chemotherapy with cisplatin and 1 cycle of radiotherapy after the operation.Metastatic lesions in the left kidney, the right lung, the first thoracic vertebrae, the retroperitoneal lymph nodes, and the mediastinal lymph nodes were identified by positron emission tomography/computed tomography 4 months after the operation, and the patient died 2 months later.PSCC is a highly malignant carcinoma, but it rarely shows a low-grade malignant histological morphology. So, efforts should be focused on differentiating it from other borderline or low malignant tumors (such as IMT) to avoid misdiagnosis.