The Alagille's syndrome consists in hypoplasia of the intrahepatic biliary ducts associated to congenital abnormalities of different organs. It is usually diagnosed in infancy due to cholestasis with good prognosis. The case of a 31-year old women who presented prominent chin, micrognathia, flattening of the nasal bone, infundibular stenosis of the pulmonary artery and cholestasis is reported. Ultrasonography demonstrated a lesion in the space of the hepatic caudate lobe with punction showing sinusoidal dilatation and infiltration of some portal spaces by lymphocytes, eosinophils and neutrophils. Samples of liver tissue obtained during laparotomy showed an absence of intrahepatic biliary ducts in the right and left lobes and preservation of those of the caudate lobe, which was also increased in size with a pseudotumoral appearance. The patients was asymptomatic with slight anicteric cholestasis at 16 months of diagnosis. The rarity of these forms of Alagille's syndrome with areas free of hypoplasia of the intrahepatic biliary ducts are of note.