Severe metabolic acidosis is common among critically ill patients, and topiramate is a rare cause that may fail recognition. We report a lady with acute encephalopathy who had severe non-anion gap metabolic acidosis that served as the clue leading to suspicion and diagnosis of topiramate toxicity and was confirmed by elevated blood topiramate levels. Additionally, we provide a review of literature on all reported cases of topiramate toxicity. Keywords: Topiramate, metabolic acidosis, migraine, altered mental status, carbonic anhydrase.Includes bibliographical reference