Undifferentiated embryonal sarcoma of the liver (UESL) is a malignant mesenchymal tumor that occurs typically in children and rarely in adults. Here we describe a case of UESL in a 51-year-old woman who presented with a cystic lesion in the liver. Because it grew slowly, the anterior segment of the liver was resected to check the lesion. Histologically, the lesion looked like a telangiectatic hepatic adenoma. Two years after resection, the tumor recurred, and she died 3 years later due to liver failure. The autopsy revealed that these lesions were UESL with massive sinusoidal invasion, and a review of the case indicated the primary lesion was also UESL. We also confirmed these tumor cells by staining with CD56, alpha-smooth muscle actin (SMA), and adipophilin, suggesting that they have a character similar to that of stellate cells in the space of Disse. The histological result of our patient revealed atypical UESL. Therefore, UESL should be considered when a hepatic lesion with degeneration is seen, even in an adult. In addition, the immunohistochemical appearance of this case implies that UESL is perhaps derived from stellate cells or stellate cells with myofibroblast differentiation in the space of Disse.