Portal biliopathy refers to abnormalities of the entire biliary tract in patients with extrahepatic portal vein obstruction. Most of the patients are asymptomatic. However, more than 80% of patients show characteristic morphologic features on endoscopic retrograde cholangiopancreatography. Symptomatic cases usually require therapy. The proposed therapies include endoscopic dilatation and stenting of the common bile duct, portosystemic shunt with splenectomy, meso Rex bypass, and occasionally, bilioenteric bypass. In patients with failed stenting and nonshuntable anatomy, liver transplant may be the only option, provided an adequate inflow can be achieved to the graft. To the best of our knowledge, only 2 cases have been reported, and these have undergone deceased-donor liver transplant for portal biliopathy. We report an index case treated successfully with living-donor liver transplant.