Abstract: Objective To elucidate the the clinical features，and the principles in diagnosis and treatment of hepatic perivascular epithelioid cell tumor（PEComa）.Methods The clinical data of 8 cases with PEComas from January，2010 to December 2018，were studied．We analyzed the clinical characters，imaging manifestation，treatment and prognosis of these cases with hepatic PEComa.Results Among the 8 patients，4 had single liver tumor，3 had multiple liver and kidney tumors，and 1 had multiple liver and kidney tumors.Four patients underwent right lobe tumor resection，three underwent left lobe tumor resection，and one underwent bilateral lobe tumor resection.Operation time was 180-310min，average（255.0±15.7）min，peroperative bleeding was 100-400ml，average（258.8±32.6）ml.All the 8 cases were confirmed as PEComa by pathology.Postoperative follow-up showed no recurrence.Conclusion Hepatic PEComa is a relatively rare liver tumor，which is definitively diagnozed by post-operative pathological examination.Surgical resection is an optimal therapy for hepatic PEComa.The major of hepatic PEComa is benign tumor，so the prognosis is relatively well.

Key words: perivascular epithelioid cell tumor, immunohistochemistry;surgical treatment;prognosis