Caroli’s disease is a congenital hepatobiliary disease named after Jacques Caroli in 1958. It was believed to involve a total or partial arrest of the remodeling of the ductal plate of the larger intrahepatic bile ducts, which was a form of ductal plate malformation. The disease was estimated to have a prevalence of one in 1,000,000. Males and females are equally affected, and more than 80% of patients present with symptoms before 30 years of age. Cholangitis is the major mode of presentation, reported in 64% of patients. The diagnosis of the disease is made with a cholangiogram of typical appearance, showing multifocal segmental dilatation. A cholangiogram can be obtained with different techniques, namely, endoscopic retrograde cholangiopancreatography (ERCP), magnetic resonance cholangiopancreatography (MRCP), or percutaneous transhepatic cholangiography (PTC). Most patients can have the diagnosis established by combining ultrasonography, computed tomography (CT) studies, and cholangiogram. The differential diagnosis for patients exhibiting the symptoms of Caroli’s disease includes primary sclerosing cholangitis (PSC), recurrent pyogenic cholangitis (RPC), polycystic liver disease, choledochal cysts, biliary papillomatosis, and, occasionally, obstructive biliary dilatation, biliary hamartomas, etc. The treatment of Caroli’s disease depends on the clinical features and the location of the biliary abnormalities. Medical and endoscopic treatments aim to reduce and treat the severity of recurrent cholangitis, hepatic abscess, and biliary obstruction. Liver resection could be contemplated for the disease with a relatively spared part of healthy liver. Orthotopic liver transplantation should be considered in patients with a diffuse form of frequent cholangitis or secondary biliary cirrhosis. The largest series about the surgical outcomes of hepatectomy reported 88.5% 5-year overall survival in selected patients, while these patients were subjected to recurrent cholangitis in the remnant liver. Contrary to liver resection, liver transplantation is a curative therapy for Caroli disease, and results with 80.9% 5-year patient survival were reported. Purposeful follow-up and surveillance of patients with Caroli’s disease after surgery is important. For patients who have undergone resection, recurrent biliary sepsis, development of cholangiocarcinoma in the remnant liver, and progression of liver cirrhosis are common during surveillance.