Persistent hypokalemia is frequently seen in Distal Renal Tubular acidosis, which is rarely described in children. We report a case of hypokalemic hyperchloremic metabolic acidosis due to distal RTA who was also found to have renal medullary nephrocalcinosis changes. This case report highlights the importance of considering hypokalemia and renal tubular acidosis in the differential diagnosis, which can prevent costly investigations and enable rapid clinical recovery in the affected child.