Vitamin A deficiency can occur as a result of malnutrition, malabsorption, or poor vitamin metabolism due to liver disease and night blindness might develop as the first symptom. Although there have been foreign reports about night blindness due to vitamin A deficiency which was derived from liver cirrhosis, primary biliary cirrhosis, intestinal bypass surgery or bariatric operation, it is hard to find reports about night blindness after percutaneous transhepatic biliary drainage for external bile drainage. We report a case of night blindness derived from fat-soluble vitamin A deficiency developed after long-term (18 months) external bile drainage for benign biliary stricture occurred after left hepatic lobectomy and hepaticojejunostomy due to the Klatskin tumor (IIIb). Her night blindness and low serum retinol level (0.02 mg/L) was dramatically improved after vitamin A supplementation. We recommend lipid-soluble vitamin supplementation on the case of long-term external bile drainage.