Abstract: 【Abstract】Objective To analyze retrospectively the clinical and pathological features of a case of hepatic epithelioid angiomyolipoma，with literatures review.Methods The clinical，radiological and pathological features of the case were analyzed，and the related literatures were reviewed. Results The case received left lobe hepatectomy and has been 15-months of tumor-free survival. Conclusions Hepatic epithelioid angiomyolipoma is extremely rare and difficult to diagnose before operation. Due to its malignant potential，surgical resection is recommended and close follow-up is important to confirm its biology feature. There is no agreement on chemotherapy regime for this neoplasm.

Key words: 【Key words】Liver, Tumor, Epithelioid angiomyolipoma