Budd Chiari syndrome is one of the rarest causes of liver disease in pregnancy. It may be primary (75%) due to thrombosis of hepatic vein or secondary (25%) due to compression of hepatic vein by an outside structure like tumors. It is usually characterized by hepatomegaly, ascites and abdominal pain. Disease is mostly diagnosed in young females with increased estrogen stimuli due to pregnancy and use of oral contraceptive pills. Pregnancy may worsen Budd Chiari syndrome and these patients may present with complications like acute & sub-acute liver disease, fulminant liver disease, bleeding oesophageal varices, portal hypertension, hepatic encephalopathy or liver failure. We present a case of decompensated (post shunt block) Budd Chiari syndrome in pregnancy.
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