Sarcoidotic involvement of the liver is frequent, albeit uncommonly symptomatic. Severe complications are rare, but may seldom require liver transplantation. Budd-Chiari syndrome has been described in a few patients with hepatic sarcoidosis. Herein we have reported the case of a young woman suffering from hepatic sarcoidosis who developed severe cholestasis and chronic Budd-Chiari syndrome. She successfully underwent orthotopic liver transplantation (OLT) and is asymptomatic with normal liver function at 3 years follow-up. Histopathological assessment of the liver explant demonstrated a florid granulomatous process, with involvement of the large intrahepatic veins, providing an anatomical basis for the vascular flow disturbances. This case adds further evidence that liver transplantation may be the curative treatment for complicated sarcoidotic liver disease.