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Intrahepatic cholestasis of pregnancy represents the most common pregnancy related liver disease in pregnancy. We present the case of rare, first-trimester onset intrahepatal cholestasis with coincidence with primary biliary cholangoitis. The combination of two diseases caused severe bile acid elevation. We decided to add Rifampicin to therapy after failure of basic combination of ursodeoxycholic acid and S-adenyl methionin. We performed cesarean section in 30 weeks of pregnancy because of acute severe elevation of bile acids. Ursodeoxycholic acid was continued postpartum with normalization of bile acids.