Portal biliopathy is due to compression of the common bile duct by varicose veins constituting portal cavernoma. Usually asymptomatic, it can occasionally be responsible for jaundice or cholangitis. We report a case of portal cavernoma secondary to pylephlebitis complicating acute appendicitis, followed eleven years later by occurrence of cholestatic jaundice. Diagnosis of portal biliopathy was done by imaging and confirmed by endoscopic retrograde cholangiography with insertion of a plastic stent into common bile duct. This stent was periodically changed and allowed regression of jaundice with a 3-year follow-up. Through a review of the literature, both clinical and therapeutic characteristics of portal biliopathy were studied.