Intravascular papillary endothelial hyperplasia (IPEH) is an unusual reactive benign lesion of vascular origin caused by endothelial cell proliferation, which rarely occurs in oral cavity. IPEH appears in various forms because it has non-specific clinical presentation, so it can be misdiagnosed as a malignant lesion.Therefore, it is important to make a differential diagnosis by understanding the characteristics of this lesion.We report a case of IPEH in a 22-year-old Korean female patient, discussing about differential diagnosis and treatment. This report presents review of literature and describes a case of IPEH.