While a few studies have investigated renal manifestations associated with mitochondrial DNA (mtDNA) variants, detailed renal histopathological changes and long-term outcomes remain poorly characterized. This study reported a family in which all 5 siblings presented with hyperuricemia and chronic kidney disease (CKD); the proband died of end-stage kidney disease at 16 years of age, while both parents were unaffected. Renal histology from 3 siblings revealed multiple foci of sclerotic and atubular glomeruli, as well as focal tubular atrophy, making early pathologic diagnosis challenging.