A 22-year-old male patient presented with progressive effort intolerance of 2-years duration. Clinical findings and investigations were suggestive of Tetralogy of Fallot (TOF). In addition, there was a conspicuous difference in the pulmonary vascularity with oligemia on the left side and relative hypervascularity on the right side. The right pulmonary artery was arising from the proximal ascending aorta and the main pulmonary artery was continuing as the left pulmonary artery. The anomalous origin of a branch pulmonary artery from the aorta (AOPA) is a rare cardiac anomaly. We report this condition in association with TOF, highlighting the differential pulmonary vascularity.