Abstract
A rare congenital hepatobiliary disorder called Caroli's disease is characterized by multifocal segmental dilatation of intrahepatic bile ducts that can affect the entire liver or only specific areas of it. Coexisting conditions with Caroli's disease include autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD). ADPKD results in the development of cysts, which are tiny fluid-filled sacs, in the kidneys. Caroli's disease is considered a rare disorder, affecting a small number of individuals worldwide. The symptoms of Caroli's disease can vary from person to person and it also may overlap with other liver and biliary disorders. As a result, it may be challenging to diagnose and manage the condition due to limited awareness and expertise. Increased awareness, research, and specialized medical care are crucial in improving outcomes for individuals affected by this rare disorder. This study involves the case of a 60- year-old woman presented with abdominal pain, fever, weight loss, and jaundice. Her imaging test endoscopic retrograde cholangiopancreatography (ERCP) signifies Caroli's disease with pancreatic duct (PD) calculi and management involves supportive care with antibiotics. Antibiotics were prescribed to prevent or treat infections such as cholangitis and nutritional supplement was recommended in managing Caroli's disease. The patient underwent pancreatic stent placement and was discharged with regular follow-up. So, this case highlights the clinical and diagnostic aspects to improve disease understanding and the progression of Caroli's illness along with ADPKD.