Portal hypertensive biliopathy is described as abnormalities of the walls of the biliary tree secondary to portal hypertension. In literature it has also been named as “Cholangiopathy associated with portal hypertension”, “Portal biliopathy” and “Portal cavernoma associated cholangiopathy”. It is usually asymptomatic and found incidentally, but rarely patients do present with symptoms of abdominal pain, jaundice, asthenia and fever. Treatment is warranted in symptomatic cases only, and is dictated by the clinical manifestations and complications of the disease process. Due to presence of underlying severe portal hypertension, endoscopic biliary intervention is usually the first line of management, and is relatively safe and often sufficient. When surgery is resorted to, a porto-systemic shunt prior to biliary bypass procedure provides early relief of obstructive biliary symptoms and often precludes the need for a biliary bypass surgery. This review describes the pathophysiology, presentation, progression and management approaches to portal biliopathy.