DOI: https://doi.org/10.36347/sjmcr.2024.v12i12.003

Pages: 2014-2017

Caroli's disease is a rare congenital disorder of the bile ducts, characterized by intrahepatic segmental dilatation. It can remain asymptomatic for many years. Imaging, especially hepatobiliary MRI, plays a central role in diagnosis, identifying pathognomonic signs (Dot-sign, cyst communication with bile ducts), and monitoring complications. Treatment primarily involves surgery, ranging from partial resection to liver transplantation for diffuse forms. This article presents two cases: one involving an association with cholangiocarcinoma and another complicated by intrahepatic stones and cholangitis. These cases highlight the major complications, diagnostic approaches, and therapeutic strategies for this rare condition.