Nephrotic syndrome is a kidney disease with proteinuria, hypoalbuminemia, and edema. One rare, potentially life-threatening complication of nephrotic syndrome is posterior reversible encephalopathy syndrome (PRES). Sudden episodes of neurological symptoms such as headache, confusion, seizures, or focal neurological deficits with radiological findings of white matter abnormalities in the parietal and occipital lobes characterize it. Multiple factors predispose an individual with nephrotic syndrome to PRES, such as uncontrolled hypertension, reduced serum albumin levels, administration of drugs (cyclosporine, tacrolimus), anasarca, disturbed body fluid status and renal insufficiency. Here, we report a case of PRES in a seven-year-old girl with nephrotic syndrome who presented with high blood pressure while admitted to the ward. Her neurological symptom rapidly recovered after the control of hypertension. Recurrence of acute severe hypertension, nephrotic state (edema/ hypoalbuminemia), and renal insufficiency may lead to recurrent PRES. Thus, early treatment of trigger factors, especially of hypertension, is vital to reduce the episodes of PRES.