SummaryNodular regenerative hyperplasia of the liver (NR.HL) is an uncommon entity of unknown origin and pathogenesis. If often occurs in association with various extrahepatic diseases such as Felty’s syndrome (FS). Because of the irregular distribution of nodules throughout the liver parenchyma the diagnosis most often relies on surgical, or post-mortem specimens. We report a case of FS with portal hypertension in which the diagnosis of FR1IL was made on the basis of routine diagnostic liver biopsy. The pathogenesis and treatment of the disease are discussed with respect to data in the literature.