Multifocal epithelioid hemangioendothelioma of the liver is a rare primary tumor with a variable course of disease. A case is presented of a 27-year-old female patient with multiple hepatic lesions on ultrasonography, suspect of metastatic tumor of the liver. Serum tumor markers were not elevated, while clinical examination of the lungs, gastrointestinal and gynecologic systems did not confirm the presence of a primary tumor process. Metastatic tumor and primary hepatocellular tumor were ruled out by fine needle aspiration cytology. Along with a characteristic immunophenotype of the vascular cell endothelium (positive for CD31 and CD34), high proliferation demonstrated by the analysis of argyrophilic nucleolar organization regions (AgNOR) and DNA aneuploidy, cytomorphological pattern suggested the diagnosis of angiosarcoma. Histopathologic finding corresponded to epithelioid hemangioendothelioma. Ten years after orthotopic liver transplantation, the patient is free from disease relapse, with regular follow up testing. Hemangioendothelioma of the liver is characterized by multifocality, which excludes resection; thus, liver transplantation is the method of choice. Therefore, preoperative diagnostic workup is of utmost importance to differentiate it from other primary and metastatic tumors of the liver.

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