Epithelioid hemangioendothelioma of the liver is a rare primary malignant tumor of vascular origin. It most frequently occurs in middle age adults and clinical findings are non-specific. Histological diagnosis may be difficult to achieve with lesions containing a large myxoid component, and immunohistochemical staining is required. A few imaging features may suggest the correct diagnosis, and radiologists should be familiar with these findings. We will illustrate the potential role of PET-CT at the time of initial presentation. Prognosis is variable and optimal management is based on a case by case analysis, ranging from clinical and imaging follow-up to liver transplantation.