Objective: To analyze and summarize the clinicopathological features, immunophenotyping, diagnosis, differential diagnosis, therapy and prognosis of papillary fibroelastoma and enhance the knowledge level about this disease by clinicians. Methods: Five cases in our hospital were analyzed retrospectively, in combined with the patients’ clinicopathological data and literature review. Paraffin-embedded tissue sections were stained by HE, immunochemistry and special histochemical staining. Results: Microscopically, papillary fibroelastoma consists of fine papillary branches. The tumor surface is covered by cubic and flat epithelium, and dense connective tissue is the main composition of papillary axis with variable mucus existing the periphery. Immunohistochemical studies revealed that the superficial tumor cells were positive for CD31, CD34 and ERG, and the central zone of papilla was focally positive for S-100. Methods of special staining showed that the stroma of the tumor expressed elastic fiber, PAS, D-PAS and AB. Conclusion: Papillary fibroelastoma is a rare primary cardiac benign tumor arising from endocardium. Although PFE has typical morphological features, it is hard for clinicians to diagnose it in lack of special clinical manifestations and symptoms. Therefore, the definite diagnosis of PFE depend on pathological examination with aid of immunohistochemistry and special histochemical staining.