Undifferentiated embryonal sarcoma of the liver is a malignancy with poor prognosis observed more frequently in children between 6 and 10 years old and very rarely found in adults. We present a case of embryonal sarcoma of the liver in a 60-year-old woman without significant medical history who presented to our attention with constitutional symptoms. Preoperative assessments did not show alterations in blood chemistry or tumor markers. Imaging studies showed a huge mass lying in the right abdominal quadrants, strictly adherent to the liver. The tumor was partially cystic with a thickened wall, sporadic contrast enhancement, and solid component. The patient underwent excision of the mass with associated liver bisegmentectomy S5-S6. Postoperative course was uneventful. The definitive histological diagnosis revealed the presence of embryonal sarcoma of the liver. We describe the clinical, histopathological, and therapeutic options adopted in the multimodal treatment of this disease.