The Alagille Syndrome (SAG), known as syndromic ductular paucity, is a multisystemic hereditary disorder of autosomal dominant transmission with a prevalence of 1/70000 live births. SAG is caused by a mutation in the Jagged1 gene (JAG1), a ligand encoding a Notch receptor that plays an important role in determining the fate of the developing cell. SAG is characterized by the association of chronic cholestasis with cardiac, ocular, skeletal and facial features. Other signs described as minor may include renal, pancreatic, and oral manifestations. The objective of our article is to provide an update on this rare syndrome and the associated oral manifestations.