Nephrotic syndrome is a common kidney disease during childhood that is characterized by alterations in glomerular filtration and leads to protein, fluid, and nutrient loss in the urine. Most patients experience peripheral, gravity-dependent edema; however, serious cases exhibit anasarca and ascites. Many long-term complications of the disease exist due to the underlying pathology and the therapies used for treatment, including metabolic bone disease, micronutrient deficiencies, and hyperlipidemia. Pharmacologic and nutrition interventions are key to appropriate management. Fluid and sodium restriction in combination with corticosteroids, albumin, and diuretics are used to manage edema. Steroid-sparing therapies like alkylating agents and calcineurin inhibitors and dietary modification to eliminate dairy and gluten may be warranted in patients with frequent relapses or steroid-refractory disease. Nutrition clinicians should familiarize themselves with the nuances of treating this disease to optimize care for children with nephrotic syndrome.