Abstract
Introduction: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of poorly understood, probably dysimmune, mechanism. It is a slow-growing disease, with a tendency to worsen. We are reporting a rare case of primary sclerosing cholangitis revealed during pregnancy. Case presentation: A 30-year-old woman who had pruritus during 12 weeks of gestation with a disturbance of the liver biologic tests. Imaging investigations found a chronic liver with areas of stenosis and dilation of VBIH and right and left hepatic bile ducts suggestive of primary sclerosing cholangitis with evidence of portal hypertension. She was put under treatment by ursodeoxycholic acid and the pregnancy proceeded without maternal-fetal complications giving birth to a healthy newborn with uneventful postpartum follow-up. Conclusion: This case highlights the importance of considering pathologies other than benign intrahepatic cholestasis of pregnancy as the cause of cholestasis during pregnancy.