Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 4 3 2019 11 04 89 93 Pantea Tajik Department of Pediatric Gastroenterology, School of Medicine Semnan University of Medical Sciences, Semnan, Iran. Amir Hossein Goudarzian Student Research Committee, Mazandarدan University of Medical Sciences, Sari, Iran. 2019 01 31 2019 06 18 Introduction: Caroli syndrome is a congenital disorder characterized by multiple segmental or saccular dilatations of the intrahepatic bile ducts associated with congenital hepatic fibrosis. Case Presentation: A 3-year-old boy with abdominal distention was referred to gastroentrology ward of Amiralmomenin hospital (Semnan, Iran) in summer 2018. In his abdominal sonography, multiple cysts were detected in the liver with hepatomegaly, and the portal vein pressure was 10 mm. Also, in liver biopsy, dilated portal bile ducts (trichrome stain) with inspissated bile and congenital hepatic fibrosis were reported. He was discharged after conservative therapy and followed up. Conclusion: Definitive treatment, i.e surgery, should be offered to prevent future complications. https://crcp.tums.ac.ir/index.php/crcp/article/view/255 https://crcp.tums.ac.ir/index.php/crcp/article/download/255/323