PEComas are an uncommon group of mesenchymal neoplasms that exhibit perivascular epithelioid cell differentiation. The term PEComa includes a collection of different subcategories, such as lymphangioleimyomatosis, clear cell tumor of the lung, and angiomyolipoma (AML) which is the topic of discussion in this article. The main problem concerning the diagnosis of hepatic AML is the wide non-specific imaging findings, stressing the need for a tissue diagnosis. Histological examination of a hepatic AML shows different types of tissues such as smooth muscle cells, fat cells (adipocytes), and blood vessels. The ultimate method for diagnosing an AML case is through immunohistochemical examination. AML displays positive immunoreactivity to HMB-45 and Melan- A, and negative to CAM5.2 and AE1/AE3 as well as S100 of the melanoma. The management of hepatic AML has been a matter of debate between different groups, and in this article we discuss a hepatic AML case that presented to our group and was treated with a minimally invasive surgical procedure.