Introduction: Congenital nephrotic syndrome (CNS) is a rare renal disorder defined by the onset of nephrotic syndrome within the first three months of life. It often presents with nonspecific clinical features, which may delay diagnosis. Early recognition is essential to initiate appropriate management and prevent complications. Case Report: A 25-day-old female neonate, the third child of consanguineous parents,presented with generalized body swelling without other significant symptoms. The antenatal and perinatal periods were uncomplicated. There was no family history of renal or liver disease. On examination, the infant had generalized edema with mild pallor and mild hepatomegaly , but no dysmorphic features. Cardiovascular examination revealed no murmurs or signs of heart failure. Initial differential diagnoses included coarctation of the aorta and liver pathology; however, echocardiography excluded coarctation, and features of liver failure subsequently resolved. Laboratory investigations demonstrated impaired renal function with +3 proteinuria, hypoalbuminemia, and hypercholesterolemia. Ultrasonography of the kidneys, ureters, and bladder revealed bilateral renal parenchymal disease. A diagnosis of congenital nephrotic syndrome was considered, and the infant was transferred to Lady Ridgeway Hospital for further management, including renal biopsy and regular intravenous albumin infusions. Discussion: CNS should be considered in neonates presenting with generalized edema, particularly in those born to consanguineous parents. Early diagnosis, genetic evaluation, and close monitoring are crucial to guide management and improve outcomes.