Background: Intravascular papillary endothelial hyperplasia, also known as Masson’s tumor, is a reactive benign lesion of vascular origin. Only few cases have been reported in the literature and the clinical features may mimic other benign and malignant lesions, making the clinical diagnosis challenging. Main observations: We report an uncommon case with detailed clinical findings affecting the oral cavity. Conclusions: We present an exceptional case of intravascular papillary endothelial hyperplasia. The oral mucosa is commonly affected by vascular lesions so it is worthy to consider intravascular papillary endothelial hyperplasia in the differential diagnosis.