Thus, this case report highlights that patients with CYP24A1 defect (a) can be challenging to diagnose, especially in CKD with secondary/tertiary hyperparathyroidism counterbalancing the effects of CYP24A1 variants, and (b) can display nephrolithiasis recurrence even after graft with a kidney with intact CYP24A1 function. The gene discussed is CYP24A1; the disease is nephrolithiasis.