Isolated partial anomalous pulmonary venous connection (PAPVC) of the entire left lung is a rare congenital anomaly with incidental diagnosis and vague symptoms, if any, until late adulthood. If left untreated, PAPVC may result in severe right ventricular failure and pulmonary vascular disease. We present the case of a 34-year-old woman with isolated PAPVC of the entire left lung. The patient underwent operation with a side-to-side left atrio-vertical vein anastomosis while on cardiopulmonary bypass and under cardioplegic arrest. She was discharged without complications and with a gradient of 2 mm Hg across the anastomosis. Left-sided PAPVC can be repaired with minimal morbidity and mortality. Surgical correction is warranted when patients are symptomatic or show evidence of right-sided overload due to unpredictability of the natural course. Recent data demonstrate that both on-pump and off-pump surgical procedures produce excellent long-term outcomes when performed without persisting gradients.