Portal biliopathy is a rare complication of extrahepatic portal vein obstruction. Jaundice occurs in symptomatic patients with fibrotic strictures. Short-term improvement in such patients can be achieved with endoscopic retrograde cholangio-pancreatography with balloon dilatation and stent placement. Surgery in these patients is traditionally two staged. We report the results of a one-stage procedure combining non-selective portal-systemic shunt surgery with biliary bypass, performed successfully on a 24-year-old man with a tight biliary stricture resulting from portal biliopathy. At 18-month follow up, the patient shows he is doing well, with normal liver function tests.