A 2-month-old infant was admitted for evaluation of jaundice. The stool had been acholic since birth. Blood test was as follows: AST/ALT 99/34 IU/L, TB/DB 12.5/8.7 mg/dL, ALP 886 IU/L, GGT 197 IU/L. Viral markers and metabolic screening tests were normal. On MRCP, an abrupt cutoff of the right proximal intrahepatic duct was noted, and biliary stasis induced parenchymal signal intensity change in the right lobe of the liver. On a hepatobiliary scintigraphy, biliary excretion to the bowel was not noted until after a 24 hour delay. Intraoperative cholangiogram (HD#7) showed abrupt cutoff of the right proximal intrahepatic duct. The open liver biopsy pathology showed paucity of interlobular bile ducts with portal and periportal fibrosis. 20% SMOF® lipid emulsion was started on HD#18. In 5 days, the stool became greenish. SMOF lipid was reduced gradually from HD#27 through HD#29. The stool became acholic again and SMOF® lipid was increased. In 4 days, the stool became greenish again. Oral ω-3 PUFA agent, Omacor® was started at HD#36, and increased gradually through HD#47. Simultaneously SMOF® lipid was decreased gradually and was stopped at HD#49. A follow-up hepatobiliary scintigraphy (HD#49) showed normal biliary excretion into the small intestine. A follow-up MRCP (HD#55) showed good visualization of the first order intra hepatic ducts in both lobes of the liver. There was no significant parenchymal signal intensity change in the right lobe of the liver. The stool remained greenish throughout the patient’s clinic follow-up for 52 weeks. we might demonstrate the effectiveness of ω-3 PUFA as a potent choleretic agent in a very rare structural pediatric hepatic dise