Glucose-6-Phosphate Dehydrogenase (G6PD)-deficiency is one of the most common enzymatic disorders of Red Blood Cells (RBCs). Cardiac surgery involving conventional Cardiopulmonary bypass (CPB) in such patients pose an increased risk of haemolysis as well as impaired oxygenation leading to prolonged ventilation. Many commonly used drugs also predispose such patients for haemolysis when they are subject to surgery. Here we describe a successful perioperative management of a 3 year old child, a known case of G6PD- deficiency who presented with a diagnosis of Atrial septal defect (ASD) with Partial Anomalous Pulmonary Venous Connection (PAPVC) and Mild-Moderate Mitral Regurgitation & underwent surgery with preplanned precautionary measures avoiding haemolysis.