Angiomyolipoma is a rare benign mesenchymal tumor of the liver. We present the case of a 32-year-old female patient that seeks medical consultation to confirm pregnancy. Ultrasound was performed and a hyperechoic lesion was detected in the left lobe of the liver. CT scan showed a heterogenic mass arising from the left lobe of the liver, fine needle aspiration biopsy was performed and diagnosis of liposarcoma was made. A laparotomy was done and a left lateral segmentectomy performed, postoperative course was uneventful. Histology and immunohistochemical analysis of the tumor revealed classical findings of primary angiomyolipoma of the liver. This case shows the difficulty often found when a preoperative diagnosis of fatty liver lesions is made. A literature review is presented and the diagnosis and management of these lesions is discussed.