Undifferentiated embryonal sarcoma of the liver (UESL) is an aggressive mesenchymal tumor, which presents a low incidence in adults. The low morbidity and atypical clinical manifestations conduce to difficulty in preoperative diagnosis. Imaging manifestations of this tumor is important for its identification. Contrast-enhanced ultrasound (CEUS) was a promising tool to characterize hepatic lesions and proved to have high diagnostic accuracy. The present study reported the CEUS characteristics of UESL in two adult patients, which exhibited peripheral rim hyper-enhancement along with progressively centripetal enhancement and large areas of perfusion defection. Sonographers should be fully aware of this rare disease for better differential diagnosis.