AIM: To retrospectively evaluate the computed tomography (CT)/magnetic resonance imaging (MRI) imaging features of epithelioid angiomyolipoma of the liver (Epi-HAML), with pathology as a reference. METHODS: The CT/MRI findings (number, diameter,lobar location, and appearance of lesions) in a series of 10 patients with 12 pathologically proven epithelioid angiomyolipomas of the liver were retrospectively analyzed. The imaging features, including attenuation/signal intensity characteristics, presence of fat,hypervascular, outer rim, and vessels within lesion,were evaluated and compared with that of non-Epi-HAML in 11 patients (13 lesions). The Fisher exact test was used to compare difference in probability of imaging features between the two types.RESULTS: For 21 patients, CT images of 15 patients and MR images of six patients were available. No patient underwent two examinations. For the 15 patients with a CT scan, all HAML lesions in the two groups (10 Epi-HAML and seven non-Epi-HAML) manifested as hypoattenuation. For the six patients with MRI, all lesions (two Epi-HAML and six non-Epi-HAML) were hypointense on T1WI (fat suppression) and hyperintense on T2WI. There were 10 non-Epi-HAML,but only two Epi-HAML lesions showed the presence of fat, which significantly different between the two types (P = 0.005). On the dynamic contrast enhancement (DCE) imaging, eight Epi-HAML, and 13 non-Epi lesions manifested as hypervascular. Punctate or curved vessels were displayed in 10 Epi-HAML as well as in nine non-Epi lesions and outer rim enhancement could be found with eight Epi-HAML as well as six non-Epi lesions.CONCLUSION: Little or no presence of adipose tissue was found to be an imaging feature of Epi-HAML, compared with the non-Epi type. In addition,hypervascularity with opacification of central punctiform or filiform vessels on DCE would be a characteristic enhancement pattern for Epi-HAML.