Epithelioid hemangioendothelioma (EH) of the liver is a rare tumor, generally considered to have low-grade malignancy. Little is known about its clinical behavior and the therapeutic strategy is not established. We report the case of a 36-year-old woman who underwent living donor liver transplantation for EH with splenic metastases and died of recurrence 8 months later. To determine if transplantation improves the prognosis of patients with EH, we must re-evaluate its indications.