Abstract : The posterior reversible encephalopathy syndrome (PRES), a rare reversible neurological condition with typical radiological findings of posterior bilateral gray and white matter abnormalities in the cerebellum and cerebral hemispheres, is increasingly being documented in paediatrics. Clinical signs and symptoms of PRES include altered mentation, drowsiness, visual abnormalities, seizures (focal or general), and non-localized headache. Here in this study, we reported two cases of children with this condition with varying presentations. We report two cases a 13-year-old male child was presented to the general ward of our hospital with swelling over the face and the lower limbs, headache, loss of appetite, and vomiting for 8 days and a 3-year-old male child was brought in for a planned MRI of the brain at our hospital after complaining of one episode of convulsion one month back. Children with new-onset seizures, a small rise in blood pressure, or hypoglycemia, and MRI findings demonstrating bilateral decreased signal intensity in the parieto-occipital areas should be suspected of PRES. To avoid misdiagnosis in these circumstances, a proper clinical and radiological correlation is required.