Origin of the left main coronary artery from the pulmonary trunk is an extremely rare, fatal, but potentially treatable anomaly. Left ventricular perfusion with desaturated blood with low pressure from the pulmonary artery results in early death. Associated pulmonary hypertension can help to prevent ischemia. We present a four-month-old boy with this anomaly associated with patent ductus arteriosus and pulmonary hypertension.