Background: Undifferentiated embryonal sarcoma of the liver (UESL) is an aggressive mesenchymal tumor that usually occurs in children. When it occurs in adults, it poses a diagnostic challenge for the medical team. The clinical manifestations are not specific, imaging studies show a hepatic mass with a mixed component (solid and cystic). To date, surgical resection with neoadjuvant therapy has yielded satisfactory results in terms of survival. Case: We present the case of a 19-year-old patient, which, to our knowledge is the only case reported in Central America, which was surgically resected and received adjuvant chemotherapy. The patient has been followed for 34 months, and she remains free of disease. Conclusion: The presented case highlights the need to raise awareness about UESL in the Central American region and promote a comprehensive diagnostic and therapeutic approach to improve disease-free survival in these patients.