BackgroundPrimary mucosa-associated lymphoid tissue (MALT) lymphoma is a rare malignancy. We found only 8 cases of MALT lymphoma in literature.Case PresentationWe report here another case of primary prostatic MALT lymphoma which is presented by hematuria and diagnosed primarily as BPH. Immunohistochemistry studies demonstrate the diagnosis and MALT lymphoma. Six months after starting the treatment the patient was alive and well.ConclusionsProstatic MALTomas are mainly presented with urinary obstruction or hematuria and have an indolent growth with a good prognosis.