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Berry Syndrome is a rare combination of congenital cardiac abnormalities firstly reported in 1982.1 It consists of aortopulmonary window, anomalous origin of the right pulmonary artery (RPA) from ascending aorta, intact ventricular septum,interruption of the aortic arch with patent ductus arteriosus(PDA). This is the 26th case reported in literature2 and the first report in Hong Kong. Delayed recognition can result in potential lethal condition.This report demonstrated the importance of prompt clinical recognition, timely echocardiography and early operation in the management of this rare cardiac anomaly.