Abstract: Gitelman syndrome (GS) is a genetically transmitted tubulopathy. It is caused by mutation in the thiazide-sensitive Na-Cl cotransporter–coding gene, SLC12A3. In this case study, we have discussed a patient diagnosed with GS and referred with muscular weakness and cramping complaints due to discontinuing potassium replacement in the follow-up. The patient was diagnosed with rhabdomyolysis and acute kidney injury secondary to hypokalemia upon determination of 2.14 mEq/L potassium, 27.610 U/L creatine kinase, and 3.09 mg/dL creatinine in further examination. Therefore, NaCl 100 cc/h isotonic was administered to the patient in addition to oral and intravenous potassium replacement. The dose of given acetazolamide was 2×250 due to the presence of severe metabolic alkalosis. Clinical and laboratory findings were fully restored to normal levels one week following the initiation of treatment.