Caroli's disease is associated with the combination of congenital dilation of the intrahepatic bile ducts, polycystic disease of the kidney and congenital hepatic fibrosis. The disease entity was first reported by Caroli and associates(1958) and was extensively reviewed in a monograph by Caroli and Corcos (1964). The original first description of Carolis disease is characterized by congenital saccular dilation of the intrahepatic bile ducts, a high incidence of stone formation and cholangitis, absence of hepatic fibrosis and portal hypertension but associated with renal tubular ectasia or other forms of cystic disease of the kidneys, possibly of the pancreas. However the pure form, as described above, has been uncommon. We have experience with a case of general anesthesia for a 12 year old female patient who had Caroli's disease, which was confirmed preoperatively by computerized tomography in Kyung Hee Medical Center. As the Carolis disease may involve hypofunction of the liver and kidney, anesthetics with less action on the liver and kidney are-preferable. This patient was premedicated with atropine and meperidine. Anesthesia was induced with thiopental and succinylcholine for intubation, then maintained with N₂O-O₂ pancuronium and meperidine. The anesthetic and immediate postoperative course was uneventful, but the patient expired with expected cardiac arrest one week later.