Critical congenital heart disease (CCHD) represents a severe subset of congenital heart defects (CHD) with high neonatal mortality rates if undiagnosed or untreated. This review highlights the prevalence, diagnosis, and management strategies for CCHD. In Asia, CHD prevalence is among the highest globally, contributing significantly to neonatal mortality. Early detection, such as prenatal ultrasound and pulse oximetry screening, plays a crucial role in improving patient outcomes. While fetal echocardiography provides diagnostic insights, it has limitations, emphasizing the value of postnatal pulse oximetry as a cost-effective, non-invasive screening tool. Management involves early interventions, including hemodynamic stabilization, pharmacological therapies like prostaglandin infusion, and surgical or catheter-based procedures. Definitive corrective surgeries, although complex, are increasingly performed in neonates to minimize long-term complications. Early diagnosis and timely referral to advanced facilities remain vital to reducing mortality and enhancing the quality of life for affected neonates.