Intravascular papillary endothelial hyperplasia is considered to be an unusual form of thrombus organization that is marked by an excessive papillary endothelial proliferation. This lesion has the propensity to occur in the skin and the subcutis. Occurrence in the gastrointestinal tract is very rare. The authors report an exceptional case of a 20-year-old young woman with intravascular papillary endothelial hyperplasia in the jejunum. The patient was referred to the hospital with a 1-week history of melena. The lesion did not recur after surgery. Histopathological examination revealed a papillary endothelial hyperplasia with an underlying arteriovenous malformation.