Hepatic angiomyolipoma is a rare entity. In this report, we describe the unusual MR findings of an angiomyolipoma with minimal fat content with histopathology correlation. This tumor had signal intensity features on various MR sequences including intense heterogeneous enhancement on immediate postgadolinium spoiled gradient echo images that resembled the appearance of a well-differentiated primary hepatocellular carcinoma. Angiomyolipoma with minimal fat content should be considered in the differential diagnosis of tumors with early diffuse heterogeneous enhancement on contrast enhanced images.