Abstract
Portal hypertensive biliopathy (PHB) is described as abnormalities of the walls of the biliary tree secondary to portal hypertension. Gastrointestinal bleeding caused by PHB is rare. PHB as a cause of serious bleeding after sphincteroplasty during ERCP is extremely rare. Here, we report a case of PHB in a young Saudi male with cell sickle anemia who developed massive hemorrhage during ERCP after balloon dilation of the ampulla of Vater. We further discussed the diagnosis and management. To the best of our knowledge, no such case has been reported.