Background <p>Distal renal tubular acidosis (dRTA) is an uncommon renal tubular disorder marked by impaired distal nephron acid excretion, resulting in hyperchloremic metabolic acidosis, persistent alkaline urine, and significant potassium loss. Complications include growth failure and rickets in children, while adults may present with osteomalacia, polyuria. Nephrolithiasis and nephrocalcinosis can be presenting feature of pediatric and adult population. Hypokalaemia, a frequent and clinically significant feature of dRTA, varies in severity from mild asymptomatic disturbances to life-threatening condition. Muscular weakness, proximal myopathy, and quadriparesis may occur in severe cases. Profound hypokalaemia may rarely precipitate life-threatening cardiac arrhythmias, including cardiac arrest.</p> Case <p>We describe a 37-year-old male who presented with acute quadriparesis due to severe hypokalaemia (serum potassium 1.3 mmol/L). While attempts at potassium correction were underway, he suffered five episodes of cardiac arrest over a span of 45&#xa0;min, each followed by successful resuscitation. Once stabilised and the potassium climbed to safe levels, a metabolic evaluation confirmed dRTA. Timely recognition and targeted electrolyte correction ensured full recovery.</p> Conclusion <p>Although hypokalaemia in dRTA is usually mild and manageable with oral or parenteral potassium therapy, it can occasionally be severe and life-threatening, as illustrated in this case. Repeated cardiac arrest due to profound hypokalaemia is the highlight of this report and emphasises the need for prompt identification and aggressive management of dRTA to prevent fatal complications.</p>