Caroli’s disease is a congenital disorder characterized by a
segmental and sacular dilatation of intrahepatic biliary ducts.
The diagnosis of Caroli’s disease depends on demonstrating
that the cystic lesions are in continuity with the biliary tree which
can be shown by ultrasonography, computerized tomography,
endoscopic retrograde cholangiopancreatography or magnetic
resonance cholangiopancreatography which is considered as
the gold standard diagnostic tool. Treatment of Caroli’s disease
relies on the location of the biliary abnormalities, while the
localized forms can be treated with surgery; liver transplantation
is the only effective modality for diffuse forms as in our current
case which reported in our Gastroenterology and Hepatology
department at Damietta cardiology and gastroenterology center
for 27 years old male presented with right upper quadrant pain,
jaundice with repeated vomiting.