RESUMO
BACKGROUND: Plasmablastic lymphoma (PBL) is a relatively new entity, classified by WHO as a rare variant of diffuse large B cell lymphoma. The present case report introduces a 17 year old girl with chronic diarrhea, abdominal pain, intra-abdominal venous thromboses, ascites, mesenteric lymphadenopathies and small intestinal polyposis, the pathologic and immunohistochemistric examinations of the polypoid lesions were in favor of PBL. Numerous cases of PBL have been reported, but to our knowledge, this is the first report of PBL presenting as small intestinal polyposis.Among lymphomas, only mantle cell lymphoma and follicular cell lymphoma have been previously reported to cause intestinal polyposis. This report introduces Plasmablastic lymphoma, a rare variant of diffuse large B cell lymphoma, as a possible cause of small intestinal polyposis.