Hepatic angiomyolipoma (AML) is a rare mesenchymal tumour with an undetermined malignant potential. Clinical symptoms are non-specific. The radiological hallmarks are high vascularization of lesion and presence of macroscopic fat. The proportion of fatty tissue varies significantly and discrepancies between pre-operative imaging and histological findings are observed in more than 50% of cases. Visualization of the draining vein may aid in differentiation between AML and hepatocellular carcinoma with abundant fatty component. Biopsy is indicated in ambiguous cases. Presence of clinical symptoms warrants surgical treatment. We present a clinical case of giant hepatic AML, discuss its typical features and treatment options.