Congenital nephrotic syndrome (CNS), a challenging form of nephrotic syndrome, is characterized by massive proteinuria, hypoalbuminemia, and edema. Extensive leakage of plasma proteins is the main feature of CNS. Patients can be diagnosed in utero or during the first few weeks of life, usually before three months. The etiology of CNS can be related to either genetic or nongenetic etiologies. Pathogenic variants in NPHS1, NPHS2, LAMB2, WT1, and PLCE1 genes have been implicated in this disease. The clinical course is complicated by significant edema, infections, thrombosis, hypothyroidism, failure to thrive, and others. Obtaining vascular access, frequent intravenous albumin infusions, diuretic use, infection prevention, and nutritional support are the mainstay management during their first month of life. The best therapy for these patients is kidney transplantation. CNS diagnosis and treatment continue to be a challenge for clinicians. This review increases the awareness about the pathogenesis, diagnosis, and management of CNS patients.