Hepatolithiasis or intrahepatic stone is associated with a variety of complications of which biliary sepsis is one. Left untreated, infection results in formation of micro-abscesses, portal thrombophlebitis and fistulation into adjacent structures. With repeated infection, biliary strictures and severe destruction of liver parenchyma occur. Biliary cirrhosis, portal hypertension and bleeding varices are the terminal manifestations. Early recognition and proper treatment are essential for the prevention of severe complications and functional deterioration.