A female infant was born at 38 weeks gestation by cesarean section. At birth, the baby had normal crying and activity. Her birth weight was 3,080 g and she did not look ill. She presented at the age of 5 days with poor feeding and associated tachypnea. She was also noted to have a holosystolic cardiac murmur. Chest radiography showed cardiomegaly and mild pulmonary plethora, more pronounced on the right. The electrocardiogram showed bilateral ventricular hypertrophy. The 64-slice multidetector computed tomography (MDCT) showed that left pulmonary artery arising normally from the main pulmonary artery but the right pulmonary artery arising directly from the right posterior aspect of the proximal ascending aorta close to the aortic valve (Figs. 1, 2). There was also a patent ductus arteriosus (PDA) and patent foramen ovale. The patient underwent surgical correction to reconnect the right pulmonary artery to the main pulmonary artery and to ligate the PDA. The operation was successful without immediate complications. However, she developed low cardiac output and acute renal failure and died on the 25th postoperative day due to progressive heart failure unresponsive to inotropic support.