Congenital nephrotic syndrome (CNS) is a rare and serious entity of renal diseases diagnosed in infants younger than three months. The triad of this syndrome is proteinuria, hypoalbuminemia, and edema. Without renal transplantation, these patients rarely live beyond the age of three years. Infections and sepsis are the most common causes of this condition among children. The majority of patients progress to end-stage renal disease early in life, even with aggressive supportive therapy. In this study, we present a case of a 10-year-old Saudi boy who had been diagnosed with CNS since he was two months old and has improved without renal transplantation.