Undifferentiated embryonal hepatic sarcoma is the third most frequent malignant hepatic neoplasm in children.
We describe two cases of school age who presented with dyspnea, weight loss, epigastric pain and a large palpable mass in the right hepatic lobe in the absence of tumor markers.
An ultrasound showed a solid mass and the computed tomography, mixed well limited cystic images. Surgical treatment was a right hemihepatectomy and the use of chemotherapy.
An undifferentiated embryonal hepatic sarcoma is a diagnostic possibility in school age children presenting with large hepatic mass and negative tumor markers. The initial treatment is a complete tumor resection and chemotherapy in order to increase survival.