The histopathology of the liver is fundamental for the differential diagnosisbetween intra- and extra- hepatic causes of neonatal cholestasis. To thehistopathologist, cholestasis means the appearance of bile within the elementsof the liver and usually associated with secondary cell injury.Aim:The aim of this study is to evaluate the role of histopathology in thediagnosis of cholestatic neonatal liver disease.Methods:The histological studies:Sections were evaluated as following:Cholestasis was graded as: Grade (1+), Grade (2+), and Grade(3+).Portal oedema was graded as: Grade (1+), Grade (2+), and Grade(3+).Proliferation of ducts graded as: Grade (1+), Grade (2+), and Grade(3+).Cholangitis: was graded as: Grade (1+), Grade (2+), and Grade(3+).Fibrosis was scored as follows: (0), (1+), (2 +), (3 +) and (4 +).Results:NH was found to be the commonest cause of neonatal cholestasis. Biliaryatresia and neonatal hepatitis were diagnosed approximately in 82% ofcholestatic cases during neonatal period.Conclusion:Portal tract changes were discriminatory variables between IHCand EHC. Features supporting the diagnosis of extra-hepatic cholestasisinclude portal fibrosis, portal oedema, proliferation of interlobular bileducts, cholestasis in neoductules and cholangitis. Only extra-medullaryhematopoiesis pointed to diagnosis of intra-hepatic cholestasis.