Nodular regenerative hyperplasia (NRH) of the liver is a rare entity characterized by the presence of nodules in the hepatic parenchyma, not surrounded by fibrous septa. The pathogenesis and etiology are unknown but an association with different diseases including some hematological disorders has been described. Its association with Hodgkin's disease is infrequent. We report the case of a 63 years old man who presented symptoms and signs of portal hypertension, hepatocellular failure with progressive deterioration and death. Postmortem examination disclosed Hodgkin's disease with hepatosplenic involvement and NRH of the liver. The association of these entities could be explained by the presence of portal infiltration contributing to portal venous obliteration and leading to portal hypertension and formation of the parenchymal nodules characteristic of this entity. Other mechanisms that could cause or influence this association can not be ruled out.