ABSTRACT— Nodular regenerative hyperplasia of the liver was found at liver biopsy and at autopsy in a 61-year-old woman with severe cholestasis. Increased bilirubin levels persisted over a follow-up period of 4 years. Microscopic findings included hyperplastic hepatocytic nodules, interlobular bile duct lesions, fibrous medial and intimal thickening of small arterial branches and portal veins, and recanalization of a thrombus in a major portal branch related to an area of focal nodular hyperplasia of the liver. Bile duct and small vessel changes were prominent in the present case. Their role as contributing factors to the development of nodular regenerative hyperplasia of the liver is discussed.