With the exception of leiomyomas, soft tissue tumors of the uterine corpus are not common. This is particularly true for vascular neoplasms, with the epithelioid hemangioendothelioma being a curiosity; not more than twenty-two cases of malignant hemangioendotheliomas have been reported in the literature so far, all of which were high-grade hemangioendotheliomas (hemangiosarcomas). We present herewith a unique case of low-grade epithelioid hemangioendothelioma of the uterus in a pregnant woman aged 29 years. The clinical, histological, and immunohistochemical characteristics of this entity, together with its differential diagnosis, are discussed. 1. Introduction Leiomyomas are common uterine neoplasms, but other, benign or malignant, soft tissue tumors are very rare [1]. Epithelioid hemangioendotheliomas are even more rare [2], with only twenty-two cases having been published in the English medical literature, and these were all malignant [3–5]. We present herewith a case of uterine epithelioid hemangioendothelioma of low-grade malignancy, the first reported in the English language literature, hoping that it will contribute to the general knowledge within the spectrum of epithelial vascular neoplasms. 2. Case Presentation A twenty-nine-year-old pregnant woman, at 32-month of gestation, was admitted to the Department of Obstetrics and Gynecology, because of premature rupture of fetal membranes, which led to urgent cesarean delivery. Physical examination did not reveal other pathological findings. Patient’s personal history was unremarkable, having no previous surgery or use of hormonal drugs. The patient was nulliparous and had no abortions. During surgery, a submucosal endometrial mass was detected in the region of isthmus, which was taken as a leiomyoma. Excision of the tumor was performed and was sent to the Department of Pathology for histopathologic evaluation. 3. Pathologic Examination Macroscopic examination of the excised specimen showed a relatively circumscribed tumor, 1.5？cm in diameter, of soft consistency. Formalin-fixed, paraffin-embedded tissue sections were stained with hematoxylin and eosin and assessed immunohistochemically for the following markers: CD31 (DAKO, clone JC70A, dilution 1？:？20), Vimentin (DAKO, clone V9, dilution 1？:？100), CD34 (NOVOCASTRA, clone QBEnd/10, dilution 1？:？50), SMA (NOVOCASTRA, clone asm-1, dilution 1？:？50), Myosin (DAKO, clone SMMS-1, dilution 1？:？70), Desmin (NOVOCASTRA, clone DE-R-11, dilution 1？:？70), Cytokeratin (DAKO, clone MNF116, dilution 1？:？70), CD10 (DAKO, clone 56C6, dilution 1？:？100), EMA
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