Bland-White-Garland syndrome (BWGS) is a rare congenital coronary artery malformation. In adult patients with BWGS, left coronary artery is supplied by collateral vessels from dilated right coronary artery. When high-pressure coronary flow drains into the low-pressure pulmonary artery with little ventricle perfusion, it causes a "coronary steal". In this study, a 53-year-old man with untreated BWGS receiving choledochotomy under general anesthesia was presented. The patient suffered from chronic biliary calculi, atrial fibrillation, complete left bundle branch block, and chronic heart failure. The anesthetic management for choledochotomy in this patient presented a special challenge. Moreover, relevant literature search was performed for all the case reports of BWGS published in PubMed and MEDLINE from 1990 to 2018. In addition, a summary of underlying pathophysiology and anesthetic implications of patients with BWGS was provided.