Background and Aims: Perivascular epithelioid cell neo-plasms (PEComas) are a rare type of mesenchymal neo-plasm and their preoperative diagnosis is challenging. In this study, we summarized the experience from a single medical center to study the examinations, clinical presen-tations, and pathological and histological characteristics of PEComas in the liver in order to optimize overall un-derstanding of the diagnosis and treatment of these neo-plasms. Methods: We conducted a retrospective analysis to investigate the clinical and pathological characteristics as well as imaging presentations of 75 patients diagnosed with hepatic PEComa in The First Affiliated Hospital of Zhe-jiang University between April 2010 and April 2020. Re-sults: Among the 75 patients, 52 were women, and the median age was 48 years. Most patients had no specific symptoms, and two were admitted to the hospital for a second time owing to relapse. All patients underwent surgi-cal resection. Histologically, 38 patients had classical angio-myolipoma (AML) and 37 had epithelioid AML. The PECo-mas were accompanied by positive immunohistochemical expression of HMB45, Melan-A, and smooth muscle actin. Follow-up data were obtained from 47 of the total 75 pa-tients, through October 2020. Two patients had metastasis after surgery. Conclusions: AML is the most common type of hepatic PEComa. There are no specific symptoms of he-patic PEComa, and serological examinations and imaging modalities for accurate preoperative diagnosis are lacking. Epithelioid AML should be considered a tumor of uncertain malignant potential; however, the prognosis of PEComa af-ter resection is promising.