Objective: Epithelioid hemangioendothelioma (EH) is a rare neoplasm usually presenting in soft tissues. About twenty cases of EH have been reported in the mediastinum. Most of these mediastinal EH exhibited an indolent course. Results: A 21-year-old woman presented with focally encapsulated mass, 5.5×4 cm in size, in the anterior mediastinum. The tumour of bone density was adhered to the lung tissue and occluded left subclavian vein. Microscopically, the tumor consisted of foci of so-called blister cells typical for EH, anastomosing cords of small epithelioid cells embedded in myxohyaline matrix with haphazardly distributed metaplastic bone and hemorrhagic cellular stroma. Spindling of the tumor cells was prominent. The tumor cells demonstrated moderate atypia, but mitotic figures were not found. Immunohistochemically, cells of primary tumor were positive for Fli1, vimentin, CD31 and CD34. Focal staining with EMA was observed. Multiple metastases were subsequently found in the liver and lungs. Metastatic deposits had the same morphology as a primary tumor.