Background: Though epileptic seizures are common in posterior reversible encephalopathy syndrome (PRES), status epilepticus (SE) as the presenting feature is rare. Objective: To study the clinical spectrum and outcome of patients with SE as presenting feature of PRES. Methods: This is a retrospective study. PRES was diagnosed based on the clinical features and imaging findings on brain MRI (n = 40) which became normal after 6 months follow-up imaging. Patients with SE as the initial manifestation of PRES were identified. Baseline information regarding the clinical presentation, etiology, past history of illness, treatment history, imaging findings, EEG and long-term clinical outcome. Result: Seizure was the most common presentation seen in 31 patients (77.5%). The etiologies in PRES were preeclampsia, or eclampsia [n = 33 (82.5%)], hypertensive encephalopathy [n = 3 (7.5%)], systemic lupus erythematosus (SLE), AIP, and chronic renal failure (CRF) in one patient each [n = 01 (2.5%)]. Brain MRI showed the involvement of parieto-occipital lobes (n = 33 [82.5%]) mostly. Status epilepticus (generalized convulsive) was the presenting feature in eight cases (20%). Among them, five cases (0.5%) had a history of chronic epilepsy. In the remaining three patients, SLE and acute intermittent porphyria, CRF precipitated the SE. Conclusion: The study highlights the clinico-etiological spectrum of PRES and the identification of SE within its context leading to the early diagnosis and management if treated early. The role of antenatal care is important for the identification and treatment of etiologies, blood pressure, proper antiepileptic drug compliance and appropriate counseling.