Objective: To investigate the clinical features, histopathological characteristics, and molecular pathological characteristics of pulmonary angiomatoid fibrous histiocytoma (AFH). Methods: One case of pulmonary AFH was collected, light microscopy observation, immunohistochemical staining and EWSR1 gene detection were performed and the literature were reviewed. Results: Under microscopy, there were incomplete fibrous pseudocapsule and unequal amounts of lymphocytes and plasma cell infiltrated around the tumor tissues, and formed lymphatic sleeve. Pseudo-vascular lacunae scattered in the tumor tisses. The tumor cells were swirling, buddle, or fasciculate arrangement, the nuclei were round or oval, some tumor cells with atypia, but rare mitotic and necrosis were detected. The immune phenotype: tumor cells Vimentin and CD99 diffuse positive, Ki-67 proliferation index was 5%. Conclusion: Angiomatoid fibrous histiocytoma is a rare borderline tumor and its diagnosis mainly depends on the pathological morphological features, and immunohistochemical markers and FISH EWSR1 detection help diagnosis and differential diagnosis; locally enlarged resection and postoperative follow-up of patients are the main principles.