eng Science and Education Publishing American Journal of Medical Case Reports 2374-216X 2018-07-28 6 7 124 127 10.12691/ajmcr-6-7-1 AJMCR2018671 article Satyajit -Mallick 1 Hasanat MA 1 Fariduddin M 1 Tahseen - Mahmood 1 Mohona -Zaman 1 Sharmin -Jahan sharmindmc@yahoo.com 1 Department of Endocrinology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh Bartter syndrome is a rare autosomal recessive renal tubulopathy resulting in hypokalemic, hypochloremic metabolic alkalosis with hyperreninemia. It consists of a set of closely related overlapping syndromes having variable presentation. Advances in molecular diagnostics have revealed the underlying mutations in numerous genes that affect the function of ion channels and transporters which normally mediate salt reabsorption in the distal nephron segments. The classic manifestations are childhood onset of fatigue, polyuria, polydipsia, salt craving, vomiting, dehydration, short stature, and failure to thrive. It should be suspected in any young, normotensive individual presenting with persistent hypokalemic alkalosis in the absence of surreptitious diuretic abuse. It requires high index of clinical suspicion for appropriate diagnosis and timely treatment have been shown to reverse the clinical and biochemical abnormalities thereby improve the outcome. Here we report a 19-year-old boy with classical Bartter syndrome that was treated with Potassium supplementation, NSAID, spironolactone and he showed significant improvement on subsequent follow-up. http://pubs.sciepub.com/ajmcr/6/7/1/ajmcr-6-7-1.pdf hypokalemia tubulopathy Bartter syndrome Gietlman syndrome