Caroli syndrome (CS) is characterized by multiple intrahepatic biliary cysts which are continuous with the biliary tract. First case is a 1-year-old, Munchkin cat that presented with icterus. This patient was diagnosed with CS based on imaging and histopathology. With surgery and supportive care, the patient has been well managed. Second case is a 1-year-old, mixed breed dog that presented with anorexia and abdominal distention. This patient was suspected to have CS based on ultrasonography, and died after 7 days. Although unusual, CS could be considered as a differential diagnosis for young animals with hepatic cysts.
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