Introduction: Methanol intoxication is an infrequent cause of poisoning in the United Sates. It can present with prominent stroke-like features and acute kidney injury. Despite the life-threatening nature of this condition, it is poorly identified by clinicians. We aim to present a case of rapidly progressive mental decline and renal involvement, discuss a diagnostic work-up and provide a critical review on therapeutic strategies. Case presentation: A 24-year-old patient presented to the emergency department with acute encephalopathy and diffuse muscular rigidity. His studies were relevant for severe anion gap metabolic acidosis, extensive brain infarction, and acute kidney injury. After excluding infections, drug intoxication, and environmental exposure, his serum methanol levels were found to be high. The patient was effectively treated with renal replacement therapy and survived with residual neurological sequelae. Conclusions: Methanol intoxication should be in the differential diagnosis of patients with brain infarction and high anion gap metabolic acidosis. Early use of renal replacement therapy may be life-saving and should be tailored on an individual basis.