Introduction: The Budd-Chiari syndrome is an obstruction of the hepatic venous drainage. It is not common as a complication of hepatic sarcoidosis but the association is possible. We report a case of Budd-Chiari syndrome complicating a hepatic sarcoidosis. Case Report: A 32-year-old female patient with a history of mediastino-pulmonary, hepatic, and splenic sarcoidosis since her young age, that stopped her medication, was readmitted at our Emergency Department for recurrent threatening hematemesis. The oesogastroduodenal fibroscopy found esophageal varices. Biological assessment found a cytolysis and cholestasis. Hepatic Doppler ultrasound showed nodular bumpy hepatomegaly, with outflow obstruction of the hepatic veins, and a laminated aspect of the inferior vena cava that remains permeable. Abdominal computed tomography (CT) showed multinodular liver and spleen, multiple lymphadenopathies, and a lack of individualization of hepatic veins. Chest CT showed an interstitial syndrome associated with bilateral mediastinal and hilar adenopathies. Viral serologies B and C, and the search for thrombophilia were negative. Hepatic sarcoidosis was previously confirmed by liver biopsy, with a negative tuberculosis testing and a high level of angiotensin-converting enzyme. All these assessments led to the diagnosis of Budd-Chiari syndrome complicating hepatic sarcoidosis. Conclusion: Budd-Chiari syndrome is thus an exceptional complication, but possible in case of hepatic sarcoidosis. Therefore, it is