Abstract: Portal biliopathy (PB) can involve the whole biliary system and this article mainly introduces extrahepatic PB that involves the extrahepatic bile duct, the cystic duct, and the gallbladder. This disease is relatively rare in clinical practice, and most doctors do not have enough knowledge of this disease. Since this disease involves the rich vascular systems and the fine biliary system, its therapeutic regimens should be selected carefully; improper therapeutic regimens may easily cause massive hemorrhage and bile duct injury, make patients' conditions worse, and even threaten their lives. At present, there is still no standard flow chart for the treatment of PB around the world. Endoscopic therapy and surgical interventional treatment are commonly used minimally invasive therapies in clinical practice. This article reviews the pathogenesis of PB and minimally invasive therapies currently used in clinical practice, in order to provide a basis for scientific diagnosis and treatment of this disease.