Epithelioid hemangioendothelioma is a rare vascular sarcoma commonly occurring in the liver, soft tissues, and organs of the chest cavity. This study provides a detailed description of the clinical presentation, diagnosis, and treatment of epithelioid hemangioendothelioma of the ovary in a 71-year-old patient. According to the pathohistological examination, a mesenchymal tumor of the ovary with epithelioid cells was diagnosed. The immunohistochemical tests confirmed the diagnosis of epithelioid hemangioendothelioma. This clinical case is of interest for clinical practitioners due to the rare location of the tumor in female reproductive organs, which has not been previously documented in the medical literature.