Background: Chronic kidney disease is widely recognized as a significant public health concern. Kidney stones are infrequently implicated as the primary cause of end-stage renal disease. Case Report: We report a case of chronic kidney disease and concomitant unilateral kidney stone with ipsilateral non-functioning kidney which surprisingly regresses after unilateral simple nephrectomy. Conclusion: Removal of the non-functioning hydronephrotic kidney with kidney stone should be kept in mind in the presence of renal failure.