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We report a young girl with classic Bartter disease (type III) with severe hypokalemia (< or = 2.0 mmol/l) who developed a prolonged heart rate-corrected QT interval of 510 ms (upper reference 430 ms) and ST segment depression in all leads. Holter electrocardiography was performed (with a plasma potassium level of 2.0 mmol/l) and it disclosed a stable sinus rhythm, a prolonged correct QT interval, more-evident ST segment depression during an increase in heart rate, a few single premature ventricular complexes, and nocturnal conduction abnormalities such as second-degree atrioventricular block 2:1. In the light of these results, the treatment was modified by increasing indomethacin from 1.5 to 3 mg/kg per day and adding spironolactone at a dose of 5 mg/kg per day. After 10 days, plasma potassium levels increased to 2.7 mmol/l and electrocardiographic abnormalities regressed. No other cardiac abnormalities were noted when the serum potassium was maintained > 2.5 mmol/l. In conclusion, this case report supports the link between arrhythmic events and chronic renal hypokalemic alkalosis in renal tubular disorders. We highlight the importance of standardizing the use of rest electrocardiography and 24-h Holter monitoring to diagnose arrhythmic events in children with severe hypokalemic renal disorders, especially in those with a plasma potassium < 2.5 mmol/l. The importance of beginning early medical treatment, to improve plasma potassium levels and reverse cardiac abnormalities, is emphasized.