Solution: Primary sclerosing cholangitis is linked to inflammatory bowel disease, but the relationship is not fully understood and in fact the underlying cause of PSC is not really known. It is an inflammatory process that leads to damage to and scarring of the bile ducts in the liver. The typical appearance, as seen in this case is of "beading" of the bile ducts. It is associated with infectious cholangitis, cirrhosis, and cholangiocarcinoma. These patients are closely followed for evidence of cholangiocarcinoma with CA19-9 levels and repeat ERCP's with brushing as well as cross sectional imaging with CT and MRI. When the disease progresses, these patients may undergo liver transplantation. Note that in this case, the patient has evidence of severe cirrhosis and portal hypertension as well and this patient had a liver transplantation within 6 months.