RESUMEN
Polycystic kidney disease (PKD) is a genetic disorder leading to end-stage renal disease more commonly in the fourth to sixth decades of life. Cyst formation in the kidneys and other organs such as the liver and pancreas is the main characteristic of this disease. A significant number of patients with PKD undergo kidney transplantation and receive significant immunosuppression, predisposing them to comorbidities such as infections and malignancies. The link between these cystic syndromes and Caroli disease (which is radiologically demonstrated as bile duct ectasia, segmental cystic dilation of intrahepatic bile ducts, with a normal common bile duct and absence of hepatic fibrosis or portal hypertension), is extremely important. Suspicion, screening, and timely diagnosis of the presence of Caroli disease in patients with PKD prior or post receiving a kidney transplant will reduce morbidity in these patients and possibly prolong both graft and patient survival. We describe a patient with autosomal dominant polycystic kidney disease who underwent recurrent admissions for presumed cholangitis and was eventually diagnosed with Caroli disease.