Introduction: Epithelioid hemangioendothelioma is a rare, locally aggressive vascular tumor, originating from soft tissue, bone, skin, and organs such as the liver or lung, exceptionally located in the oral cavity. Most of the cases of oral epithelioid hemangioendothelioma are asymptomatic, and diagnosis is hampered by the fact that the histological features are somewhat between hemangioma and angiosarcoma, with epithelioid cells, intracytoplasmic vacuoles, low mitotic activity, and (rarely) necrosis. Immunohistochemical analysis is required to rule out carcinoma or other epithelioid vascular neoplasms. Case presentation: We present a rare case of a 59-year-old Caucasian male patient with oral epithelioid hemangioendothelioma for which clinical and cytological diagnosis was difficult, in spite of the patient’s history. The lesion was nonspecific, mimicking ulcerative stomatitis, but histological and immunohistochemical evaluation finally managed to establish the right diagnosis. Subsequently, the patient underwent surgical excision of the lesion followed by oncological treatment — chemotherapy. Conclusions: Although at first examination seemed that another lesion has developed, rigorous histology and immunohistochemistry tests proved the presence of epithelioid hemangioendothelioma, a very rare entity located in the oral cavity, which required a proper surgical and oncological approach.