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Forty-nine studies of the systematic review were included, yielding 105 cases. The literature showed similarity in all variables. Despite the uncommon frequency, clinicians and oral pathologists should familiarize themselves with the similarities between intravascular papillary endothelial hyperplasia and some other lesions in terms of clinical and histological features. Despite the uncommon frequency, clinicians and oral pathologists should familiarize themselves with the similarities between intravascular papillary endothelial hyperpl