Case Presentation: A 13-month-old child with past medical history of congenital adrenal insufficiency presented to the emergency department with vomiting and diarrhea. Initially the child was noticed to have bradycardia with normal blood pressure. An electrocardiogram (ECG) showed tall T waves, broad QRS complex, and widened PR interval suggestive of severe hyperkalemia. The initial blood gas showed potassium of 10.7 millimoles per liter. The patient was started on calcium gluconate with immediate resolution of ECG changes. Further management with insulin, dextrose, and sodium olystyrene sulfonate led to normal potassium levels. Discussion: Hyperkalemia is a life-threatening condition in children, especially in those with congenital adrenal insufficiency. The ECG showed different changes as the levels of serum potassium levels increased ranging from tall T waves, wide QRS complex, increased PR interval to arrythmias. Immediate treatment with calcium gluconate in such cases has significant cardioprotective effect. It is important to recognize the ECG changes manifested by changes in serum potassium levels. Our patient had classic ECG changes manifested in severe hyperkalemia.