Background: Presentation of seizures in patients with chronic obstructive pulmonary disease (COPD) is an infrequent finding. Posterior reversible encephalopathy is a rare and potentially treatable entity in such scenarios. Case description: We present a case of a 61-year-old female, with a diagnosed case of COPD for 5 years, on regular treatment. She was admitted to the intensive care unit (ICU) with a history of fever, vomiting, and an increased level of drowsiness. She had shown good improvement with the initial management of acute infective exacerbation of COPD. After 96 hours of admission, she had status epilepticus. Magnetic resonance imaging (MRI) findings and clinical features solved the mystery of status epilepticus and altered sensorium. Presentation of posterior reversible encephalopathy syndrome (PRES) in acute exacerbation of COPD is a rare condition but should be kept in mind as one of the important differential diagnoses for those presenting with seizures with or without encephalopathy, even in the absence of accelerated hypertension. Neuroimaging is essential for the exclusion of other possible conditions. PRES is treatable and reversible with or without full recovery. Conclusion: Seizures in patients with COPD can be caused by PRES, which is a potentially reversible condition. A conclusive diagnosis can be established by relevant neuroimaging. Clinical significance: The PRES is a potential cause of seizures in patients with COPD.