While renal angiomyolipomas (AMLs) are benign and fat-rich, renal epithelioid AMLs (EAMLs) have a low fat content and demonstrate aggressive behavior. Herein, we present the case of a 42-year-old female with a fat-rich renal tumor that was resected due to its large size and the risk of bleeding. Pathological findings suggested AML with focal epithelioid differentiation and mild atypia. Even though the tumor did not meet the current diagnostic criteria for EAML, the patient developed hepatic EAML four years later. The hepatic EAML demonstrated similar imaging characteristics to those of hepatocellular carcinoma (HCC). To the best of our knowledge, there is a limited body of literature documenting malignant behavior displayed by a fat-rich renal AML with focal epithelioid differentiation.