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Introduction: Hyponatremia is one of the most common electrolyte disorders in hospitalized patients. Sodium < 120 mEq/l, accounts for one-third of all hyponatremia cases with a hospital mortality ranging from 15-25% and 1 year mortality exceeding 20%. Survivors are at risk for permanent neurological sequelae. Profound hyponatremia < 105 mEq/L is exceedingly rare, with a reported prevalence of less than 0.1% in inpatient settings. We present a case of a profound hyponatremia with serum sodium of 101 mmol/L associated with altered mental status and seizure induced by beer potomania. Description: A 64 year old female with history of chronic alcohol use (4-5 cans of beer daily). Presented to the Emergency Department (ED) with vomiting, and altered mental status. In the ED she experienced one episode of generalized tonic-clonic seizure. Initial labs showed sodium level of 101 mmol/L, with chloride level of < 60, low serum osmolality (240 mOsm/kg), high anion gap metabolic acidosis (AG>30), ethanol level of >60 mg/dL, urine chloride of 31 and urine sodium of 47. The patient was admitted to the intensive care unit,nephrology consulted and hypertonic saline 3% was administered followed by isotonic fluid. Sodium level increased to 116 mmol/L over 24 hours which raised concerns for overcorrection, as a result desmopressin (DDAVP) and D5W were started to safely re-correct serum sodium over several days to prevent osmotic demyelination. The sodium level stabilized and reached 134 mmol/L at the 6th day of hospitalization with marked clinical improvement and she was subsequently discharged in stable condition. Discussion: Beer potomania is a syndrome caused by excessive alcohol intake with little dietary solute intake, impairing renal free water excretion and leading to significant dilutional hyponatremia. Our Case demonstrate an extreme hyponatremia level of 101 mmol/L due to beer potomania, one of the lowest sodium levels reported in the literature. A systemic review of published case reports showed that serum sodium values between 105 and 115 mmol/L in beer potomania, with rare cases reporting values as low as 101 mmol/L or below. The treatment of severe hyponatremia requires extreme caution to the rate of the sodium correction (between 4–6 mmol/L in 24 hours) to prevent osmotic demyelination syndrome (ODS).