A 50-year-old Danish man, who neither had wasting disease nor was taking steroid-containing drugs, complained of abdominal distension, due to a markedly enlarged liver. Percutaneous needle biopsies were taken from the liver, and the findings gave suspicion of a neoplastic tumor. Because of reduced liver function and treatment-resistant ascites, he underwent liver transplantation without a definite preoperative diagnosis. The resected liver weighed 2900 g, and almost all of the parenchyma was destroyed and replaced by multicystic blood-filled spaces, diagnosed as extensive peliosis hepatis complicating liver cirrhosis. Extensive peliosis with liver cirrhosis is a rare condition. Only two cases, caused by contraceptives and treated by liver transplantation, are reported in the English-language literature. We could find no cause other than alcohol abuse lasting several years in this patient, and classified the present case as idiopathic extensive peliosis hepatis. Although scarce subjective findings and misleading liver biopsies made an exact diagnosis difficult, an orthotopic liver transplantation was the only treatment for such complicated peliosis hepatis.