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Tetralogy of Fallot (TOF) is a frequently encountered congenital heart defect, and the detailed diagnostic criteria are well established. There may be other anomalies associated with TOF, but the anomalous origin of the left pulmonary artery (LPA) from the ascending aorta (AOPA) is a rare one. The diagnosis of this anomaly, which may be mistaken for the absence of the LPA, should be made in early life to avoid unilateral pulmonary hypertension and also for appropriate surgical planning. We are reporting a case of a 16-year-old child, with the diagnosis of TOF associated with anomalous origin of the LPA from the AOPA who underwent a one-stage surgical correction of this anomaly.