A 37-year-old female had liver nodules found in an abdominal ultrasound scan. The radiological features were suggestive of hepatocellular adenomas, although there were some atypical findings. Two years later, one of the nodules showed dimensional progression and intralesional hemorrhage. The patient underwent a left hepatectomy and the postoperative course was uneventful. The histological exam and the immunohistochemistry were consistent with the diagnosis of PEComa. The PEComa is a mesenchymal tumor rarely described in the liver. The preoperative diagnosis of PEComa is very difficult and, with this report, the authors intend to increase the limited knowledge relating to natural history and optimal treatment of this rare condition.