Peliosis hepatis is a very uncommon disease defined by pathological lesions located in the liver and in the spleen characterized by blood filled lakes irregularly disseminated throughout parenchyma, without abnormalities of the hepatic veins or the vena cava system. A new case, regularly followed 3 years after the initial diagnosis is reported. Recent reports of the literature have pointed out etiologic factors, particularly iatrogenic factors. None of the reported factors could be found out in the present case. This emphasizes the need of epidemiological studies of such a rare disease (case-control study in a multicentric basis). Finally a successful diagnosis approach is proposed: transvenous (transjugular) liver biopsy. This procedure prevents hemorrhagic risk which could be a complication of routine transcutaneous liver biopsy in this disease.