Sequential neuroimaging before and after seizures has not been reported in patients with nephrotic syndrome and recurrent posterior reversible encephalopathy syndrome (PRES). We report a 13-year-old nephrotic female patient with recurrent PRES during and after cessation of cyclosporine A (CyA). She had headache, visual disturbance and acute hypertension, followed by seizures. The brain magnetic resonance imaging (MRI) before seizures revealed a high signal intensity area on fluid-attenuated inversion recovery and diffusion-weighted images and a low signal intensity area on T1-weighted images in bilateral parieto-occipital and left temporal lobes. Cessation of CyA resulted in clinical improvement. The follow-up MRI 2 months after the initial episode showed a complete resolution. Six months later, she had similar symptoms, edema, severe hypoalbuminemia, renal insufficiency, and acute pancreatitis before seizures. The brain MRI after seizures showed similar abnormalities in the same regions, which completely resolved 2 months later. Recurrence of acute severe hypertension, nephrotic state (edema/ hypoalbuminemia), and renal insufficiency may lead to recurrent PRES and thus early treatment of trigger factors, especially of hypertension, is important to reduce the episodes of PRES.