Objective To discuss the clinical characteristics of hepatic perivascularepithelioid cell tumor (PEComa). Methods Clinical data of one patient with hepatic PEComa in the Second Affiliated Hospital, Zhejiang University School of Medicine in 2011 were analyzed retrospectively. The informed consent of the patient was obtained and the ethical committee approval was received. The patient was a 25-year-old female and was admitted in hospital for the physical examination finding of space occupying lesions in the liver. The results of physical and laboratory examinations were normal. Multiple round-like mass of low density with poorly defined borders were observed in the liver by CT scan. Significant enhancement was observed in the arterial phase by enhancement scan, and homo- or hypo-enhancement in the delayed phase. The liver lesions showed intermediated signal intensity on T1WI by MRI and slightly hyperintense on T2WI. Significant enhancement was observed in the arterial phase after enhancement and degraded in the delayed phase. The patient was primarily diagnosed with liver focal nodular hyperplasia clinically. Results After sufficient preoperative preparation, hepatectomy was performed on the patient under general anesthesia by tracheal intubation on December 8th , 2011. The tumor was observed
composed of polygonal morphology cells of epithelial cells without lipocytes or abnormal blood vessels by pathological examination. The tumor was observed with strongly positive human melanoma black-45 (HMB-45), smooth muscle actin (SMA), and positive vimentin, cluster of differentiation (CD) 34. The diagnosis of hepatic PEComa was confirmed pathologically. The patient recovered well and was discharged from hospital 1 week after operation. No recurrence or metastasis was observed during the regular follow-up till the submission date. Conclusions Hepatic PEComa is extremely rare without specific clinical manifestation. The diagnosis depends on the pathological examination. Surgical resection is an effective method for the tumor with a good prognosis.