Abstract: Primary hepatic perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm, typically presenting benign or indolent biological behavior. However, its clinical and radiological manifestations are non⁃specific, often leading to misdiagnosis as hepatocellular carcinoma (HCC), hepatic adenoma or other metastatic lesions. This report describes a case of primary hepatic PEComa in a patient who presented with a hepatic space⁃occupying lesion persisting for over a year, without typical clinical or imaging features, and was initially misdiagnosed as HCC. Following multi⁃disciplinary team (MDT) consultation, the patient underwent radical hepatectomy. Postoperative pathological examination showed positive expression of SMA, HMB⁃45 and Melan⁃A, confirming epithelioid⁃type PEComa. There was no recurrence sign during the 6⁃month postoperative follow⁃up. This case underscores the importance of considering liver biopsy and immunohistochemistry in atypical cases, even when imaging suggests HCC. Furthermore, it advocates for the establishment of an individualized treatment approach centered on MDT collaboration to achieve precise therapeutic outcomes.

Key words: Liver tumor, Perivascular epithelioid cell tumor, Diagnosis, Therapeutic