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Intravascular papillary endothelial hyperplasia (IPEH) is a rare benign vascular lesion that rarely occurs in the oral cavity. Its clinical features are similar to neoplasms, which are easily identified in the oral cavity, and it can be misdiagnosed as an angiosarcoma. Therefore, it is important to recognize the characteristics of the lesion in order to both diagnose and treat it properly. We report a case of IPEH in a young Korean female patient, as well as discuss its differential diagnosis and treatment using a review of the related literature.