Left hemitruncus (aortic anomalous origin of the left pulmonary artery) is relatively rare than the right hemitruncus. Early diagnosis is critical to prevent early occurrence of pulmonary vascular obstructive disease in the left pulmonary bed in patients with tetralogy of Fallot presenting with left hemitruncus arteriosus. To the best of our knowledge, there are only five surviving cases with left hemitruncus and tetralogy of Fallot who were operated in the first decade of life in the literature. Any pediatric case undergoing staged surgery with full recovery has not been reported yet in the literature. In this article, we report a one-year-old girl with tetralogy of Fallot and left hemitruncus with a late diagnosis who underwent staged correction surgery with palliative left pulmonary artery banding followed by total repair.