Abernethy malformation, also known as congenital extrahepatic shunt, is a rare anomaly characterized by partial or complete diversion of the portal blood into the systemic venous circulation. The clinical manifestations of Abernethy malformation during childhood include neonatal cholestasis, failure to thrive, mental retardation, and other congenital defects. We report a case of Abernethy malformation Type Ⅱ in a 9-year-old boy whose left ventricle was slightly enlarged because of several major aortopulmonary collateral arteries but normal laboratory examinations five years earlier. The characteristics of congenital heart disease in patients with Abernethy malformation are discussed. We propose that enlargement of the left ventricular with systemic-pulmonary collateral circulation should raise the suspicion of Abernethy malformation.