Hepatoportal sclerosis is a rare and underdiagnosed liver condition characterised by histological alterations of the portal venules and hepatic sinusoids, with periportal fibro-sis and vascular narrowing. Its diagnosis is histological and requires liver biopsy, with the identification of specific vascular lesions often heterogeneously distributed within the liver parenchyma. Its clinical presentation is variable: in some patients, the condition is discovered incidentally due to mild, persistent elevation of transaminases; in others, it presents with signs of portal hypertension such as splenomegaly, esophageal varices and thrombocytopenia. The presence or absence of portal hypertension is the main prognostic factor: in cases without portal hypertension, the course is benign and does not require specific follow-up. Conversely, in children with portal hypertension, the disease may progress to liver failure or hepatocellular carcinoma and make systematic and long-term monitoring necessary. Its clinical management focuses on the prevention of complications related to portal hypertension. Liver transplantation is still the only curative option in advanced or complicated cases.
