Undifferentiated embryonal sarcoma of the liver (UESL) is an uncommon malignant neoplasm in the pediatric population, accounting for a small fraction of primary hepatic tumors but associated with an aggressive clinical course and poor prognosis. Surgical resection remains the mainstay of treatment; however, extended hepatectomy can leave patients with limited functional reserve and predispose them to hepatic failure, making liver transplantation (LT) a therapeutic alternative in selected cases.

We present the case of a 7-year-old female patient with UESL who initially underwent extended right hepatectomy. Despite an adequate immediate postoperative outcome, she subsequently developed progressive hepatic dysfunction and complications related to portal hypertension. Due to clinical deterioration and absence of curative options, the patient was listed for LT and successfully underwent transplantation using the classical split-liver technique. The postoperative course was favorable, with early weaning from vasoactive and ventilatory support, prompt recovery of oral intake, and hospital discharge on postoperative day ten. At present follow-up, she remains clinically stable with preserved graft function under immunosuppressive therapy.

This case highlights the role of LT as a life-saving option in pediatric patients with hepatic sarcomas who are not candidates for complete resection or who develop liver failure after major hepatectomy. Moreover, it emphasizes the importance of careful donor–recipient selection, specialized surgical expertise, and multidisciplinary follow-up to optimize outcomes in this challenging setting.

Liver neoplasms, Liver transplantation, Hepatectomy, Sarcoma, Surgical procedures, Operative, Tissue transplantation, Liver regeneration