Hepatic angiomyolipoma (HAML) is a rare benign mesenchymal tumor of the liver, which has highly variable imaging appearances, often leads to missed diagnosis and misdiagnosis. The images of 2 patients with HAML confirmed by pathology were presented in this study, and the typical imaging features of the HAML, the underlying pathophysiological mechanism, and the differential diagnosis were briefly summarized so as to deepen the understanding of HAML and to improve the diagnosis and differential diagnosis abilities of HAML, then reduce the rates of missed diagnosis and misdiagnosis of the HAML.