Posterior reversible encephalopathy syndrome (PRES) is a rare serious syndrome of central nervous system that can develop in both adults and children. It is characterised by acute onset of headache, confusion, seizures, or focal neurological deficits along with radiological abnormalities in the parietal and occipital lobes. In the past, this syndrome has been mainly described in adults but rare in children. However, it is not uncommon in paediatric nephrology. Hypertension, renal disease, immunosuppression, and chemotherapy of malignancies are triggers for PRES. Here, we report a case of 12 years old boy with steroid-sensitive nephrotic syndrome presenting as PRES.