Epithelioid hemangioendothelioma is a rare, low-grade malignant vascular tumour. It is frequently seen in the liver, but can occur in the lungs, bones, and other soft tissues. Although survival time might be reasonable in cases that can undergo liver transplantation, there is no consensus on the treatment of metastatic patients. We report a 24-year-old female patient with rapidly progressing malignant epithelioid hemangioendothelioma that presented with acute abdominal distension. The patient was refractory to anthracycline and Interferon treatment and died 6.5 months after the diagnosis.