Introduction: EAST syndrome is a rare autosomal recessive disorder characterized by epilepsy , ataxia, sensorineural deafness and renal tubulopathy . It results from mutations in the KCNJ10 gene encoding a potassium channel expressed in the brain, inner ear and distal renal tubules. The disorder typically presents in early childhood with neurological manifestations and persistent electrolyte abnormalities resembling Gitelman-like tubulopathy . Due to its rarity and variable presentation, recognition is often delayed. Case presentation: A 15-year-old girl with global developmental delay , epilepsy , cerebellar ataxia and sensorineural hearing loss presented with a respiratory tract infection, vomiting and poor oral intake. Investigations revealed severe hypokalaemia, hypomagnesaemia and hyponatraemia persisting on serial testing. Arterial blood gas analysis demonstratedmetabolic alkalosis. Electrocardiography showed changes consistent with severe hypokalaemia. She was managed with fluid resuscitation and intravenous potassium and magnesium replacement under strict monitoring. Further evaluation confirmed renalpotassium and magnesium wasting suggestive of Gitelman-like tubulopathy . She required aggressive electrolyte supplementation with spironolactone to reduce renal potassium losses. Considering her longstanding neurological features and newly recognized renal tubulopathy , EAST syndrome was suspected clinically . She was discharged on long-term oral electrolyte supplementation. Follow up showed improvement in sensorium and muscle power. Genetic confirmation is awaited. Conclusion: EAST syndrome should be suspected in patients with epilepsy , ataxia, sensorineural hearing loss and persistent renal electrolyte wasting. Early recognition and multidisciplinary management are essential to improve outcomes and quality of life.