We report an unusual case of primary cardiac epithelioid hemangioendothelioma (EHE) with atypical features, which was treated by orthoptic transplantation with a good outcome for 10 years despite recurrent pulmonary and nodal metastases. EHE is a rare vascular tumor that belongs to the group of malignant proliferations from the new World Health Organization classification of soft tissue tumors. EHE may harbor atypical features that confer a more aggressive course, albeit better than that of conventional angiosarcomas. Histological examination of the primary cardiac tumor revealed a proliferation of large epithelioid tumor cells presenting atypical features and a mitotic index of 3 mitoses per 10 high power fields. In contrast, pulmonary metastases exhibited typical features of EHE, and CD 34 and CD 31 immunostainings strongly stained cytoplasmic vascular lumen. In this report, we illustrate the potential aggressiveness of the atypical variant of EHE and suggest that transplantation might be considered as an alternative therapy in the treatment of EHE of the heart.