The Budd-Chari syndrome (BCS) is a group of disorders of hepatic vein outflow at various levels from the hepatic veins to inferior vena cave. We describe a 49-year-old man with multiple intrahepatic lesions who had been diagnosed with the BCS. The inferior vena cavography showed hepatic vein occlusion and long-range obstruction of inferior vena cava. The biopsy proved to be hyperplastic nodules, also called large regenerative nodules (LRNs). Both benign regenerative nodules and hepatocellular carcinoma (HCC) appear in patients with BCC; however, published reports about the diagnosis and differential diagnosis are limited. The incidence of HCC in patients with BCS varies greatly depending on geography. This case illustrates that benign nodules can arise in BCS patients. We reviewed published reports and speculated that medical procedures leading to portal perfusion decrease may be associated with the development of these hyperplastic nodules.