A 5-year-old girl was admitted because of an acute onset of weakness in her extremities. She had experienced a similar episode before but had recovered spontaneously. She had previously been diagnosed with distal renal tubular acidosis(RTA) at the age of 2 months. During the period of acute paralysis, her serum potassium level was 1.8 mmol/L and the muscle enzymes were markedly raised suggesting massive rhabdomyolysis. Although hypokalemia is common in renal tubular acidosis, acute paralytic presentation is uncommon and is rarely described in children. We report a case of distal RTA complicated with hypokalemic paralysis with a brief review of related literatures.