The authors consider ectopic biliary varices as a possible cause of portal biliopathy in extrahepatic portal hypertension. The main diagnostic methods including ultrasound CT, MRI, endoscopic ultrasonography, cholangioscopy, difficulties of differential diagnosis and clinical manifestations of portal biliopathy are presented. Various treatment options including portosystemic shunting and endoscopic biliary decompression are discussed. The authors emphasize the role of differentiated treatment depending on bile duct lesions. The study not only reviews this problem, but also highlights the issues requiring further analysis for selection of optimal diagnostic and treatment methods.