This eight-month-old female developed jaundice at one week of age. As the jaundice deepened, the child's bilirubin level was found to be over 30 mg/dL and the patient experienced a few convulsive episodes. A liver biopsy produced a diagnosis of extrahepatic bile duct obstruction. A subsequent exploratory laparotomy showed an absence of extrahepatic bile duct structures--including the gallbladder--and an intraoperative cholangiogram failed to show any large biliary channel. A jejunohepatostomy in the portal area was performed in the hope of establishing bile drainage and the patient was placed on the waiting list for a liver transplant. However, the patient progressively deteriorated, developed infections, and after a long turbulent course, died of pneumonia. An autopsy was performed and the diagnosis of extrahepatic bile duct obstruction was confirmed.