Von Meyenburg complexes, or multiple biliary hamartomas, are often asymptomatic lesions incidentally discovered during abdominal or hepatic imaging. The presentation of clinically significant Von Meyenburg complexes ranges from cholestasis and self-limited episodes of cholangitis to malignant degeneration into cholangiocarcinoma. In cases of persistent or recurrent cholangitis, treatment is a significant challenge. Definitive source control with liver transplantation, as in other cases of cholestatic liver disease, may be necessary.